1

Pharmacy Medical PolicyIntravenous Immunoglobulin

Table of Contents Policy: Commercial Policy History Endnotes

Policy: Medicare Information Pertaining to All Policies Forms

Coding Information References

Policy Number: 310BCBSA Reference Number: 8.01.05

Related Policies See medical policy #422, RSV Immunoprophylaxis (RSV-IVIg)

PolicyCommercial Members: Managed Care (HMO and POS), PPO, and Indemnity

Note: All requests for indications listed and not listed on the medical policy guidelines may be submittedto BCBSMA Pharmacy Operations by completing the Prior Authorization Form on the last page of thisdocument. Physicians may also submit requests for exceptions via the web using Express PAth whichcan be found on the BCBSMA provider portal or directly on the web at https://provider.express-path.com.

This medication is not covered by the pharmacy benefit. It is covered by the Medical Benefit or as aHome Infusion Therapy.

We cover intravenous immunoglobulin (IVIg) for the following diagnoses only:

Blood disorders Bone marrow transplant patients (for prevention of infection or GVH prevention)

12, 14, 32

Multiple myeloma and immunoproliferative neoplasms8

Immune neutropenia8

Multiple myeloma without mention of remission8

Multiple myeloma in remission8

Plasma cell leukemia without mention of remission8

Plasma cell leukemia in remission8

Other immunoproliferative neoplasms without mention of remission8

Other immunoproliferative neoplasms in remission8

Agranulocytosis8

Common variable immunodeficiency, severe combined immunodeficiency, Wiskott-Aldrich syndrome,and X-linked immunodeficiency

14

Prevention of infection in patients with primary defective antibody synthesis14

2

Fetal alloimmune thrombocytopenia15

Autoimmune hemolytic anemia17

Agammaglobulinemia -primary humoral immunodeficiency12,14

Hypogammaglobulinemia -primary humoral immunodeficiency12,14

Chronic lymphocytic leukemia (CLL) with frequent infections12,14

Idiopathic thrombocytopenic purpura (ITP).12,24

Infectious diseases HIV and AIDS

13

Prevention of infection in HIV-infected children12,14

Prior to solid organ transplant, treatment of patients at high risk of antibody-mediated rejection,including highly sensitized patients, and those receiving an ABO incompatible organ

Solid organ transplant recipients at risk for cytomegalovirus infections and pneumonia.7

Neurologic conditions: Guillain Barre Syndrome (GBS)

15

Chronic severe myasthenia gravis,7,22

for severe exacerbations causing disability Myasthenic crisis (i.e., an acute episode of respiratory muscle weakness) in patients with

contraindication to plasma exchange27

Myasthenia gravis in patients with chronic debilitating disease in spite of treatment withcholinesterase inhibitors, or complications from or failure of steroids and/or azathioprine.

Hereditary and idiopathic peripheral neuropathy8, 19

Peroneal muscular atrophy8, 19

Hereditary sensory neuropathy8, 19

Refsum’s disease8

Idiopathic progressive polyneuropathy8

Multiple Sclerosis: for patients with relapsing-remitting disease (not primary or secondary progressiveMS)

11,15

Chronic inflammatory demyelinating polyneuropathy15

Demyelinating polyneuropathy associated with IgM paraproteinemia20

Multifocal motor neuropathy in patients with GM1 antibodies and conduction block15

Stiff-man syndrome35

(Covered for Medicare HMO Blue and Medicare PPO Blue members only,effective 6/07/2010).

Other: Dermatomyositis/polymyositis Refractory dermatomyositis; in combination with other immunosuppressive agents Kawasaki syndrome

12,15

Pemphigus vulgaris (Effective 10/01, coverage is provided as once in a lifetime benefit only, seedosing guideline below)

9

Prior to solid organ transplant; treatment of patients at high risk of antibody-mediated rejection,including highly sensitized patients, and those receiving an ABO incompatible organ

34,Effective

January 2007 Following solid organ transplant; treatment of antibody-mediated rejection

34,Effective January 2007.

We cover intravenous immunoglobulin (IVIg) for treatment of the following biopsy-proven conditions, forour Medicare HMO Blue and Medicare PPO Blue members only, in accordance with CMS guidelines.See endnote 26 for policy guidelines:

26

Pemphigus foliaceus26

Bullous pemphigoid26

Mucous membrane pemphigoid (also known as Cicatrical pemphigoid)26

Epidermolysis bullosa acquisita.26

3

Treatment dosage varies according to indication and preparation used. The following dosage schedulehave been suggested by Medicare:

8

Primary humoral immunodeficiency 100 to 500 mg/kg IV, every monthIdiopathic thrombocytopenic purpura 2 gm/kg for induction and up to 1 gm/kg

subsequently.18

Chronic lymphocytic leukemia 100 to 500 mg/kg IV, every monthBone marrow transplant recipients 100 to 500 mg/kg IV every month. The usual

treatment for CMV prophylaxis in transplant recipientsshould not exceed 90 days.

Kawasaki syndrome 400 mg/kg per day for 5 daysGuillain-Barre syndrome 400 mg/kg per day for 5 daysChronic severe myasthenia gravis 400 mg/kg per day for 5 daysPemphigus vulgaris

182 gm/kg per course of therapy in a month and 3courses of therapy in a 6-month period.

18Coverage is

provided as once in a lifetime benefit only.

We do not cover intravenous immunoglobulin in the following conditions:

Blood disorders Acquired factor VIII inhibitors

16

Acute lymphoblastic leukemia16

Aplastic anemia16

Diamond-Blackfan anemia16

Hemophagocytic syndrome16

Nonimmune thrombocytopenia16

Red cell aplasia16

Thrombotic thrombocytopenic purpura.30

Rheumatologic diseases Behcet’s syndrome

16

Inclusion body myositis2,16

, because it does not work in this disorder Rheumatoid arthritis

4,16and other connective tissue diseases including systemic lupus erythematosus

Scleroderma10

Systemic Lupus Erythematosis1

Other vasculitides besides Kawasaki disease; including vasculitis associated with anti-neutrophilcytoplasmic antibodies (ANCA; e.g., (Wegener’s granulomatosis, polyarteritis nodosa),Goodpasture’s syndrome, and vasculitis associated with other connective tissue diseases.

16, 34

Neurologic conditions Epilepsy

16

Multiple sclerosis: primary progressive or secondary progressive types, because it has not beenshown to offer additional health benefits to patients with these types of MS

3,11,16

Paraneoplastic syndromes including but not limited to Lambert-Eaton syndrome16

Stiff-man syndrome (Non-covered for commercial products only).28

Infectious Chronic sinusitis

16

Recurrent otitis media.16

Other Adrenoleukodystrophy

16

Asthma16

Chronic fatigue syndrome16

4

Cystic fibrosis16

Diabetes mellitus16

Hemolytic uremic syndrome16

Idiopathic lumbosacral flexopathy10

Recurrent fetal loss6,16

Recurrent Spontaneous Abortion33

Bullous pemphigoid21

(except for Medicare HMO Blue and Medicare PPO Blue members above) Pemphigus foliaceus

23(except for Medicare HMO Blue and Medicare PPO Blue members above)

Cicatricial pemphigoid (mucous membrane pemphigoid)23

(except for Medicare HMO Blue andMedicare PPO Blue members above)

Epidermolysis bullosa aquisita23

(except for Medicare HMO Blue and Medicare PPO Blue membersabove)

Recurrent spontaneous pregnancy loss25

Idiopathic environmental illness29

Myasthenia gravis in patients responsive to immunosuppressive treatment30

Post-infectious sequelae30

Organ transplant rejection30

Uveitis30

Demyelinating optic neuritis30

Recent-onset dilated cardiomyopathy30

Other disorders not listed above.

CPT Codes / HCPCS Codes / ICD-9 CodesThe following codes are included below for informational purposes. Inclusion or exclusion of a code doesnot constitute or imply member coverage or provider reimbursement. Please refer to the member’scontract benefits in effect at the time of service to determine coverage or non-coverage as it applies to anindividual member. A draft of future ICD-10 Coding related to this document, as it might look today, isincluded below for your reference.

Providers should report all services using the most up-to-date industry-standard procedure, revenue, anddiagnosis codes, including modifiers where applicable.

CPT CodesThere is no specific CPT code for this service.

HCPCS CodesHCPCScodes: Code DescriptionJ0850 Injection, cytomegalovirus immune globulin intravenous (human), per vialJ1459 Injection, immune globulin (Privigen), intravenous, nonlyophilized (e.g., liquid), 500 mgJ1557 Injection, immune globulin, (Gammaplex), intravenous, nonlyophilized (e.g., liquid), 500

mgJ1559 Injection, immune globulin (Hizentra), 100 mgJ1561 Injection, immune globulin, (Gamunex/Gamunex-C/Gammaked), nonlyophilized (e.g.,

liquid), 500 mgJ1566 Injection, immune globulin, intravenous, lyophilized (e.g., powder), 500 mg [Carimune]J1568 Injection, immune globulin, (Octagam), intravenous, nonlyophilized (e.g., liquid), 500

mgJ1569 Injection, immune globulin, (Gammagard liquid), intravenous, nonlyophilized, (e.g.,

liquid), 500 mgJ1572 Injection, immune globulin, (Flebogamma/Flebogamma Dif), intravenous,

nonlyophilized (e.g., liquid), 500 mgJ1599 Injection, immune globulin, intravenous, nonlyophilized (e.g., liquid), not otherwise

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specified, 500 mg

Diagnosis codingICD-9-CMdiagnosiscodes: Code Description042 Human immunodeficiency virus [HIV] disease078.5 Cytomegaloviral disease136.1 Behcet's syndrome203.00 Multiple myeloma, without mention of having achieved remission203.01 Multiple myeloma, in remission203.02 Multiple myeloma, in relapse203.10 Plasma cell leukemia, without mention of having achieved remission203.11 Plasma cell leukemia, in remission203.12 Plasma cell leukemia, in relapse203.80 Other immunoproliferative neoplasms, without mention of having achieved remission203.81 Other immunoproliferative neoplasms, in remission203.82 Other immunoproliferative neoplasms, in relapse204.00 Acute lymphoid leukemia, without mention of having achieved remission204.01 Acute lymphoid leukemia, in remission204.02 Acute lymphoid leukemia, in relapse204.10 Chronic lymphoid leukemia, without mention of having achieved remission204.11 Chronic lymphoid leukemia, in remission204.12 Chronic lymphoid leukemia, in relapse249.00 Secondary diabetes mellitus without mention of complication, not stated as

uncontrolled, or unspecified249.01 Secondary diabetes mellitus without mention of complication, uncontrolled249.10 Secondary diabetes mellitus with ketoacidosis, not stated as uncontrolled, or

unspecified249.11 Secondary diabetes mellitus with ketoacidosis, uncontrolled249.20 Secondary diabetes mellitus with hyperosmolarity, not stated as uncontrolled, or

unspecified249.21 Secondary diabetes mellitus with hyperosmolarity, uncontrolled249.30 Secondary diabetes mellitus with other coma, not stated as uncontrolled, or unspecified249.31 Secondary diabetes mellitus with other coma, uncontrolled249.40 Secondary diabetes mellitus with renal manifestations, not stated as uncontrolled, or

unspecified249.41 Secondary diabetes mellitus with renal manifestations, uncontrolled249.50 Secondary diabetes mellitus with ophthalmic manifestations, not stated as uncontrolled,

or unspecified249.51 Secondary diabetes mellitus with ophthalmic manifestations, uncontrolled249.60 Secondary diabetes mellitus with neurological manifestations, not stated as

uncontrolled, or unspecified249.61 Secondary diabetes mellitus with neurological manifestations, uncontrolled249.70 Secondary diabetes mellitus with peripheral circulatory disorders, not stated as

uncontrolled, or unspecified249.71 Secondary diabetes mellitus with peripheral circulatory disorders, uncontrolled249.80 Secondary diabetes mellitus with other specified manifestations, not stated as

uncontrolled, or unspecified249.81 Secondary diabetes mellitus with other specified manifestations, uncontrolled249.90 Secondary diabetes mellitus with unspecified complication, not stated as uncontrolled,

or unspecified249.91 Secondary diabetes mellitus with unspecified complication, uncontrolled

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250.00 Diabetes mellitus without mention of complication, type II or unspecified type, not statedas uncontrolled

250.01 Diabetes mellitus without mention of complication, type I [juvenile type], not stated asuncontrolled

250.02 Diabetes mellitus without mention of complication, type II or unspecified type,uncontrolled

250.03 Diabetes mellitus without mention of complication, type I [juvenile type], uncontrolled250.10 Diabetes with ketoacidosis, type II or unspecified type, not stated as uncontrolled250.11 Diabetes with ketoacidosis, type I [juvenile type], not stated as uncontrolled250.12 Diabetes with ketoacidosis, type II or unspecified type, uncontrolled250.13 Diabetes with ketoacidosis, type I [juvenile type], uncontrolled250.20 Diabetes with hyperosmolarity, type II or unspecified type, not stated as uncontrolled250.21 Diabetes with hyperosmolarity, type I [juvenile type], not stated as uncontrolled250.22 Diabetes with hyperosmolarity, type II or unspecified type, uncontrolled250.23 Diabetes with hyperosmolarity, type I [juvenile type], uncontrolled250.30 Diabetes with other coma, type II or unspecified type, not stated as uncontrolled250.31 Diabetes with other coma, type I [juvenile type], not stated as uncontrolled250.32 Diabetes with other coma, type II or unspecified type, uncontrolled250.33 Diabetes with other coma, type I [juvenile type], uncontrolled250.40 Diabetes with renal manifestations, type II or unspecified type, not stated as

uncontrolled250.41 Diabetes with renal manifestations, type I [juvenile type], not stated as uncontrolled250.42 Diabetes with renal manifestations, type II or unspecified type, uncontrolled250.43 Diabetes with renal manifestations, type I [juvenile type], uncontrolled250.50 Diabetes with ophthalmic manifestations, type II or unspecified type, not stated as

uncontrolled250.51 Diabetes with ophthalmic manifestations, type I [juvenile type], not stated as

uncontrolled250.52 Diabetes with ophthalmic manifestations, type II or unspecified type, uncontrolled250.53 Diabetes with ophthalmic manifestations, type I [juvenile type], uncontrolled250.60 Diabetes with neurological manifestations, type II or unspecified type, not stated as

uncontrolled250.61 Diabetes with neurological manifestations, type I [juvenile type], not stated as

uncontrolled250.62 Diabetes with neurological manifestations, type II or unspecified type, uncontrolled250.63 Diabetes with neurological manifestations, type I [juvenile type], uncontrolled250.70 Diabetes with peripheral circulatory disorders, type II or unspecified type, not stated as

uncontrolled250.71 Diabetes with peripheral circulatory disorders, type I [juvenile type], not stated as

uncontrolled250.72 Diabetes with peripheral circulatory disorders, type II or unspecified type, uncontrolled250.73 Diabetes with peripheral circulatory disorders, type I [juvenile type], uncontrolled250.80 Diabetes with other specified manifestations, type II or unspecified type, not stated as

uncontrolled250.81 Diabetes with other specified manifestations, type I [juvenile type], not stated as

uncontrolled250.82 Diabetes with other specified manifestations, type II or unspecified type, uncontrolled250.83 Diabetes with other specified manifestations, type I [juvenile type], uncontrolled250.90 Diabetes with unspecified complication, type II or unspecified type, not stated as

uncontrolled250.91 Diabetes with unspecified complication, type I [juvenile type], not stated as uncontrolled250.92 Diabetes with unspecified complication, type II or unspecified type, uncontrolled250.93 Diabetes with unspecified complication, type I [juvenile type], uncontrolled277.00 Cystic fibrosis without mention of meconium ileus

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277.01 Cystic fibrosis with meconium ileus277.02 Cystic fibrosis with pulmonary manifestations277.03 Cystic fibrosis with gastrointestinal manifestations277.09 Cystic fibrosis with other manifestations277.86 Peroxisomal disorders279.00 Hypogammaglobulinemia, unspecified279.01 Selective IgA immunodeficiency279.02 Selective IgM immunodeficiency279.03 Other selective immunoglobulin deficiencies279.04 Congenital hypogammaglobulinemia279.05 Immunodeficiency with increased IgM279.06 Common variable immunodeficiency279.12 Wiskott-aldrich syndrome279.3 Unspecified immunity deficiency283.0 Autoimmune hemolytic anemias283.11 Hemolytic-uremic syndrome284.01 Constitutional red blood cell aplasia284.81 Red cell aplasia (acquired)(adult)(with thymoma)284.89 Other specified aplastic anemias284.9 Aplastic anemia, unspecified287.31 Immune thrombocytopenic purpura287.32 Evans' syndrome287.49 Other secondary thrombocytopenia287.5 Thrombocytopenia, unspecified288.09 Other neutropenia288.4 Hemophagocytic syndromes333.91 Stiff-man syndrome335.10 Spinal muscular atrophy, unspecified335.11 Kugelberg-Welander disease335.19 Other spinal muscular atrophy335.21 Progressive muscular atrophy337.00 Idiopathic peripheral autonomic neuropathy, unspecified337.01 Carotid sinus syndrome337.09 Other idiopathic peripheral autonomic neuropathy340 Multiple sclerosis345.00 Generalized nonconvulsive epilepsy, without mention of intractable epilepsy345.01 Generalized nonconvulsive epilepsy, with intractable epilepsy345.10 Generalized convulsive epilepsy, without mention of intractable epilepsy345.11 Generalized convulsive epilepsy, with intractable epilepsy345.2 Petit mal status345.3 Grand mal status345.40 Localization-related (focal) (partial) epilepsy and epileptic syndromes with complex

partial seizures, without mention of intractable epilepsy345.41 Localization-related (focal) (partial) epilepsy and epileptic syndromes with complex

partial seizures, with intractable epilepsy345.50 Localization-related (focal) (partial) epilepsy and epileptic syndromes with simple partial

seizures, without mention of intractable epilepsy345.51 Localization-related (focal) (partial) epilepsy and epileptic syndromes with simple partial

seizures, with intractable epilepsy345.60 Infantile spasms, without mention of intractable epilepsy345.61 Infantile spasms, with intractable epilepsy345.70 Epilepsia partialis continua, without mention of intractable epilepsy345.71 Epilepsia partialis continua, with intractable epilepsy

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345.80 Other forms of epilepsy and recurrent seizures, without mention of intractable epilepsy345.81 Other forms of epilepsy and recurrent seizures, with intractable epilepsy345.90 Epilepsy, unspecified, without mention of intractable epilepsy345.91 Epilepsy, unspecified, with intractable epilepsy356.2 Hereditary sensory neuropathy356.3 Refsum's disease356.4 Idiopathic progressive polyneuropathy356.8 Other specified idiopathic peripheral neuropathy356.9 Unspecified hereditary and idiopathic peripheral neuropathy357.0 Acute infective polyneuritis357.81 Chronic inflammatory demyelinating polyneuritis357.82 Critical illness polyneuropathy358.00 Myasthenia gravis without (acute) exacerbation358.01 Myasthenia gravis with (acute) exacerbation358.30 Lambert-Eaton syndrome, unspecified358.31 Lambert-Eaton syndrome in neoplastic disease358.39 Lambert-Eaton syndrome in other diseases classified elsewhere364.3 Unspecified iridocyclitis377.30 Optic neuritis, unspecified382.00 Acute suppurative otitis media without spontaneous rupture of eardrum382.01 Acute suppurative otitis media with spontaneous rupture of eardrum382.02 Acute suppurative otitis media in diseases classified elsewhere382.1 Chronic tubotympanic suppurative otitis media382.2 Chronic atticoantral suppurative otitis media382.3 Unspecified chronic suppurative otitis media382.4 Unspecified suppurative otitis media382.9 Unspecified otitis media425.4 Other primary cardiomyopathies446.1 Acute febrile mucocutaneous lymph node syndrome [MCLS]446.21 Goodpasture's syndrome446.4 Wegener's granulomatosis446.6 Thrombotic microangiopathy447.6 Arteritis, unspecified473.0 Chronic maxillary sinusitis473.1 Chronic frontal sinusitis473.2 Chronic ethmoidal sinusitis473.3 Chronic sphenoidal sinusitis473.8 Other chronic sinusitis473.9 Unspecified sinusitis (chronic)486 Pneumonia, organism unspecified493.00 Extrinsic asthma, unspecified493.01 Extrinsic asthma with status asthmaticus493.02 Extrinsic asthma with (acute) exacerbation493.10 Intrinsic asthma, unspecified493.11 Intrinsic asthma with status asthmaticus493.12 Intrinsic asthma with (acute) exacerbation493.20 Chronic obstructive asthma, unspecified493.21 Chronic obstructive asthma with status asthmaticus493.22 Chronic obstructive asthma with (acute) exacerbation493.81 Exercise induced bronchospasm493.82 Cough variant asthma493.90 Asthma,unspecified type, unspecified493.91 Asthma, unspecified type, with status asthmaticus

9

493.92 Asthma, unspecified type, with (acute) exacerbation530.81 Esophageal reflux629.81 Recurrent pregnancy loss without current pregnancy646.30 Recurrent pregnancy loss, unspecified as to episode of care or not applicable646.31 Recurrent pregnancy loss, delivered, with or without mention of antepartum condition646.33 Recurrent pregnancy loss, antepartum condition or complication694.4 Pemphigus694.5 Pemphigoid710.0 Systemic lupus erythematosus710.1 Systemic sclerosis710.3 Dermatomyositis710.4 Polymyositis714.0 Rheumatoid arthritis714.1 Felty's syndrome714.2 Other rheumatoid arthritis with visceral or systemic involvement714.30 Polyarticular juvenile rheumatoid arthritis, chronic or unspecified714.31 Polyarticular juvenile rheumatoid arthritis, acute714.32 Pauciarticular juvenile rheumatoid arthritis714.33 Monoarticular juvenile rheumatoid arthritis729.1 Myalgia and myositis, unspecified757.39 Other specified anomalies of skin780.71 Chronic fatigue syndrome996.80 Complications of transplanted organ, unspecified996.81 Complications of transplanted kidney996.82 Complications of transplanted liver996.83 Complications of transplanted heart996.84 Complications of transplanted lung996.85 Complications of transplanted bone marrow996.86 Complications of transplanted pancreas996.87 Complications of transplanted intestine996.88 Complications of transplanted organ, stem cell996.89 Complications of other specified transplanted organV08 Asymptomatic human immunodeficiency virus [HIV] infection statusV42.0 Kidney replaced by transplantV42.1 Heart replaced by transplantV42.6 Lung replaced by transplantV42.7 Liver replaced by transplantV42.83 Pancreas replaced by transplantV42.84 Organ or tissue replaced by transplant, intestinesV42.89 Other specified organ or tissue replaced by transplantV42.9 Unspecified organ or tissue replaced by transplant

Facility codingICD-9-CMprocedurecodes: Code Description99.14 Injection or infusion of immunoglobulin

ICD-10 Diagnosis CodesICD-10-CMDiagnosiscodes: Code DescriptionB20 Human immunodeficiency virus [HIV] disease

10

B25.0 Cytomegaloviral pneumonitis

B25.1 Cytomegaloviral hepatitis

B25.2 Cytomegaloviral pancreatitis

B25.8 Other cytomegaloviral diseases

B25.9 Cytomegaloviral disease, unspecified

C88.2 Heavy chain disease

C88.3 Immunoproliferative small intestinal disease

C88.8 Other malignant immunoproliferative diseases

C88.9 Malignant immunoproliferative disease, unspecified

C90.00 Multiple myeloma not having achieved remission

C90.01 Multiple myeloma in remission

C90.02 Multiple myeloma in relapse

C90.10 Plasma cell leukemia not having achieved remission

C90.11 Plasma cell leukemia in remission

C90.12 Plasma cell leukemia in relapse

C90.20 Extramedullary plasmacytoma not having achieved remission

C90.21 Extramedullary plasmacytoma in remission

C90.22 Extramedullary plasmacytoma in relapse

C90.30 Solitary plasmacytoma not having achieved remission

C90.31 Solitary plasmacytoma in remission

C90.32 Solitary plasmacytoma in relapse

C91.00 Acute lymphoblastic leukemia not having achieved remission

C91.01 Acute lymphoblastic leukemia, in remission

C91.02 Acute lymphoblastic leukemia, in relapse

C91.10 Chronic lymphocytic leukemia of B-cell type not having achieved remission

C91.11 Chronic lymphocytic leukemia of B-cell type in remission

C91.12 Chronic lymphocytic leukemia of B-cell type in relapse

D59.0 Drug-induced autoimmune hemolytic anemia

D59.1 Other autoimmune hemolytic anemias

D59.3 Hemolytic-uremic syndrome

D60.0 Chronic acquired pure red cell aplasia

D60.1 Transient acquired pure red cell aplasia

D60.8 Other acquired pure red cell aplasias

D60.9 Acquired pure red cell aplasia, unspecified

D61.01 Constitutional (pure) red blood cell aplasia

D61.1 Drug-induced aplastic anemia

D61.2 Aplastic anemia due to other external agents

D61.3 Idiopathic aplastic anemia

D61.89 Other specified aplastic anemias and other bone marrow failure syndromes

D61.9 Aplastic anemia, unspecified

D69.3 Immune thrombocytopenic purpura

D69.41 Evans syndrome

D69.59 Other secondary thrombocytopenia

D69.6 Thrombocytopenia, unspecified

D70.8 Other neutropenia

D76.1 Hemophagocytic lymphohistiocytosis

D76.2 Hemophagocytic syndrome, infection-associated

D80.0 Hereditary hypogammaglobulinemia

D80.1 Nonfamilial hypogammaglobulinemia

D80.2 Selective deficiency of immunoglobulin A [IgA]

D80.3 Selective deficiency of immunoglobulin G [IgG] subclasses

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D80.4 Selective deficiency of immunoglobulin M [IgM]

D80.5 Immunodeficiency with increased immunoglobulin M [IgM]

D82.0 Wiskott-Aldrich syndrome

D83.0Common variable immunodeficiency with predominant abnormalities of B-cell numbersand function

D83.2 Common variable immunodeficiency with autoantibodies to B- or T-cells

D83.8 Other common variable immunodeficiencies

D83.9 Common variable immunodeficiency, unspecified

D84.8 Other specified immunodeficiencies

D84.9 Immunodeficiency, unspecified

E08.00Diabetes mellitus due to underlying condition with hyperosmolarity without nonketotichyperglycemic-hyperosmolar coma (NKHHC)

E08.01 Diabetes mellitus due to underlying condition with hyperosmolarity with coma

E08.10 Diabetes mellitus due to underlying condition with ketoacidosis without coma

E08.11 Diabetes mellitus due to underlying condition with ketoacidosis with coma

E08.21 Diabetes mellitus due to underlying condition with diabetic nephropathy

E08.22 Diabetes mellitus due to underlying condition with diabetic chronic kidney disease

E08.29 Diabetes mellitus due to underlying condition with other diabetic kidney complication

E08.311Diabetes mellitus due to underlying condition with unspecified diabetic retinopathy withmacular edema

E08.319Diabetes mellitus due to underlying condition with unspecified diabetic retinopathywithout macular edema

E08.321Diabetes mellitus due to underlying condition with mild nonproliferative diabeticretinopathy with macular edema

E08.329Diabetes mellitus due to underlying condition with mild nonproliferative diabeticretinopathy without macular edema

E08.331Diabetes mellitus due to underlying condition with moderate nonproliferative diabeticretinopathy with macular edema

E08.339Diabetes mellitus due to underlying condition with moderate nonproliferative diabeticretinopathy without macular edema

E08.341Diabetes mellitus due to underlying condition with severe nonproliferative diabeticretinopathy with macular edema

E08.349Diabetes mellitus due to underlying condition with severe nonproliferative diabeticretinopathy without macular edema

E08.351Diabetes mellitus due to underlying condition with proliferative diabetic retinopathy withmacular edema

E08.359Diabetes mellitus due to underlying condition with proliferative diabetic retinopathywithout macular edema

E08.36 Diabetes mellitus due to underlying condition with diabetic cataract

E08.39Diabetes mellitus due to underlying condition with other diabetic ophthalmiccomplication

E08.40 Diabetes mellitus due to underlying condition with diabetic neuropathy, unspecified

E08.41 Diabetes mellitus due to underlying condition with diabetic mononeuropathy

E08.42 Diabetes mellitus due to underlying condition with diabetic polyneuropathy

E08.43 Diabetes mellitus due to underlying condition with diabetic autonomic (poly)neuropathy

E08.44 Diabetes mellitus due to underlying condition with diabetic amyotrophy

E08.49Diabetes mellitus due to underlying condition with other diabetic neurologicalcomplication

E08.51Diabetes mellitus due to underlying condition with diabetic peripheral angiopathywithout gangrene

E08.52Diabetes mellitus due to underlying condition with diabetic peripheral angiopathy withgangrene

E08.59 Diabetes mellitus due to underlying condition with other circulatory complications

12

E08.610 Diabetes mellitus due to underlying condition with diabetic neuropathic arthropathy

E08.618 Diabetes mellitus due to underlying condition with other diabetic arthropathy

E08.620 Diabetes mellitus due to underlying condition with diabetic dermatitis

E08.621 Diabetes mellitus due to underlying condition with foot ulcer

E08.622 Diabetes mellitus due to underlying condition with other skin ulcer

E08.628 Diabetes mellitus due to underlying condition with other skin complications

E08.630 Diabetes mellitus due to underlying condition with periodontal disease

E08.638 Diabetes mellitus due to underlying condition with other oral complications

E08.641 Diabetes mellitus due to underlying condition with hypoglycemia with coma

E08.649 Diabetes mellitus due to underlying condition with hypoglycemia without coma

E08.65 Diabetes mellitus due to underlying condition with hyperglycemia

E08.69 Diabetes mellitus due to underlying condition with other specified complication

E08.8 Diabetes mellitus due to underlying condition with unspecified complications

E08.9 Diabetes mellitus due to underlying condition without complications

E09.00Drug or chemical induced diabetes mellitus with hyperosmolarity without nonketotichyperglycemic-hyperosmolar coma (NKHHC)

E09.01 Drug or chemical induced diabetes mellitus with hyperosmolarity with coma

E09.10 Drug or chemical induced diabetes mellitus with ketoacidosis without coma

E09.11 Drug or chemical induced diabetes mellitus with ketoacidosis with coma

E09.21 Drug or chemical induced diabetes mellitus with diabetic nephropathy

E09.22 Drug or chemical induced diabetes mellitus with diabetic chronic kidney disease

E09.29 Drug or chemical induced diabetes mellitus with other diabetic kidney complication

E09.311Drug or chemical induced diabetes mellitus with unspecified diabetic retinopathy withmacular edema

E09.319Drug or chemical induced diabetes mellitus with unspecified diabetic retinopathywithout macular edema

E09.321Drug or chemical induced diabetes mellitus with mild nonproliferative diabeticretinopathy with macular edema

E09.329Drug or chemical induced diabetes mellitus with mild nonproliferative diabeticretinopathy without macular edema

E09.331Drug or chemical induced diabetes mellitus with moderate nonproliferative diabeticretinopathy with macular edema

E09.339Drug or chemical induced diabetes mellitus with moderate nonproliferative diabeticretinopathy without macular edema

E09.341Drug or chemical induced diabetes mellitus with severe nonproliferative diabeticretinopathy with macular edema

E09.349Drug or chemical induced diabetes mellitus with severe nonproliferative diabeticretinopathy without macular edema

E09.351Drug or chemical induced diabetes mellitus with proliferative diabetic retinopathy withmacular edema

E09.359Drug or chemical induced diabetes mellitus with proliferative diabetic retinopathywithout macular edema

E09.36 Drug or chemical induced diabetes mellitus with diabetic cataract

E09.39 Drug or chemical induced diabetes mellitus with other diabetic ophthalmic complication

E09.40Drug or chemical induced diabetes mellitus with neurological complications withdiabetic neuropathy, unspecified

E09.41Drug or chemical induced diabetes mellitus with neurological complications withdiabetic mononeuropathy

E09.42Drug or chemical induced diabetes mellitus with neurological complications withdiabetic polyneuropathy

E09.43Drug or chemical induced diabetes mellitus with neurological complications withdiabetic autonomic (poly)neuropathy

E09.44 Drug or chemical induced diabetes mellitus with neurological complications with

13

diabetic amyotrophy

E09.49Drug or chemical induced diabetes mellitus with neurological complications with otherdiabetic neurological complication

E09.51Drug or chemical induced diabetes mellitus with diabetic peripheral angiopathy withoutgangrene

E09.52Drug or chemical induced diabetes mellitus with diabetic peripheral angiopathy withgangrene

E09.59 Drug or chemical induced diabetes mellitus with other circulatory complications

E09.610 Drug or chemical induced diabetes mellitus with diabetic neuropathic arthropathy

E09.618 Drug or chemical induced diabetes mellitus with other diabetic arthropathy

E09.620 Drug or chemical induced diabetes mellitus with diabetic dermatitis

E09.621 Drug or chemical induced diabetes mellitus with foot ulcer

E09.622 Drug or chemical induced diabetes mellitus with other skin ulcer

E09.628 Drug or chemical induced diabetes mellitus with other skin complications

E09.630 Drug or chemical induced diabetes mellitus with periodontal disease

E09.638 Drug or chemical induced diabetes mellitus with other oral complications

E09.641 Drug or chemical induced diabetes mellitus with hypoglycemia with coma

E09.649 Drug or chemical induced diabetes mellitus with hypoglycemia without coma

E09.65 Drug or chemical induced diabetes mellitus with hyperglycemia

E09.69 Drug or chemical induced diabetes mellitus with other specified complication

E09.8 Drug or chemical induced diabetes mellitus with unspecified complications

E09.9 Drug or chemical induced diabetes mellitus without complications

E10.10 Type 1 diabetes mellitus with ketoacidosis without coma

E10.11 Type 1 diabetes mellitus with ketoacidosis with coma

E10.21 Type 1 diabetes mellitus with diabetic nephropathy

E10.22 Type 1 diabetes mellitus with diabetic chronic kidney disease

E10.29 Type 1 diabetes mellitus with other diabetic kidney complication

E10.311 Type 1 diabetes mellitus with unspecified diabetic retinopathy with macular edema

E10.319 Type 1 diabetes mellitus with unspecified diabetic retinopathy without macular edema

E10.321Type 1 diabetes mellitus with mild nonproliferative diabetic retinopathy with macularedema

E10.329Type 1 diabetes mellitus with mild nonproliferative diabetic retinopathy without macularedema

E10.331Type 1 diabetes mellitus with moderate nonproliferative diabetic retinopathy withmacular edema

E10.339Type 1 diabetes mellitus with moderate nonproliferative diabetic retinopathy withoutmacular edema

E10.341Type 1 diabetes mellitus with severe nonproliferative diabetic retinopathy with macularedema

E10.349Type 1 diabetes mellitus with severe nonproliferative diabetic retinopathy withoutmacular edema

E10.351 Type 1 diabetes mellitus with proliferative diabetic retinopathy with macular edema

E10.359 Type 1 diabetes mellitus with proliferative diabetic retinopathy without macular edema

E10.36 Type 1 diabetes mellitus with diabetic cataract

E10.39 Type 1 diabetes mellitus with other diabetic ophthalmic complication

E10.40 Type 1 diabetes mellitus with diabetic neuropathy, unspecified

E10.41 Type 1 diabetes mellitus with diabetic mononeuropathy

E10.42 Type 1 diabetes mellitus with diabetic polyneuropathy

E10.43 Type 1 diabetes mellitus with diabetic autonomic (poly)neuropathy

E10.44 Type 1 diabetes mellitus with diabetic amyotrophy

E10.49 Type 1 diabetes mellitus with other diabetic neurological complication

E10.51 Type 1 diabetes mellitus with diabetic peripheral angiopathy without gangrene

14

E10.52 Type 1 diabetes mellitus with diabetic peripheral angiopathy with gangrene

E10.59 Type 1 diabetes mellitus with other circulatory complications

E10.610 Type 1 diabetes mellitus with diabetic neuropathic arthropathy

E10.618 Type 1 diabetes mellitus with other diabetic arthropathy

E10.620 Type 1 diabetes mellitus with diabetic dermatitis

E10.621 Type 1 diabetes mellitus with foot ulcer

E10.622 Type 1 diabetes mellitus with other skin ulcer

E10.628 Type 1 diabetes mellitus with other skin complications

E10.630 Type 1 diabetes mellitus with periodontal disease

E10.638 Type 1 diabetes mellitus with other oral complications

E10.641 Type 1 diabetes mellitus with hypoglycemia with coma

E10.649 Type 1 diabetes mellitus with hypoglycemia without coma

E10.65 Type 1 diabetes mellitus with hyperglycemia

E10.69 Type 1 diabetes mellitus with other specified complication

E10.8 Type 1 diabetes mellitus with unspecified complications

E10.9 Type 1 diabetes mellitus without complications

E11.00Type 2 diabetes mellitus with hyperosmolarity without nonketotic hyperglycemic-hyperosmolar coma (NKHHC)

E11.01 Type 2 diabetes mellitus with hyperosmolarity with coma

E11.21 Type 2 diabetes mellitus with diabetic nephropathy

E11.22 Type 2 diabetes mellitus with diabetic chronic kidney disease

E11.29 Type 2 diabetes mellitus with other diabetic kidney complication

E11.311 Type 2 diabetes mellitus with unspecified diabetic retinopathy with macular edema

E11.319 Type 2 diabetes mellitus with unspecified diabetic retinopathy without macular edema

E11.321Type 2 diabetes mellitus with mild nonproliferative diabetic retinopathy with macularedema

E11.329Type 2 diabetes mellitus with mild nonproliferative diabetic retinopathy without macularedema

E11.331Type 2 diabetes mellitus with moderate nonproliferative diabetic retinopathy withmacular edema

E11.339Type 2 diabetes mellitus with moderate nonproliferative diabetic retinopathy withoutmacular edema

E11.341Type 2 diabetes mellitus with severe nonproliferative diabetic retinopathy with macularedema

E11.349Type 2 diabetes mellitus with severe nonproliferative diabetic retinopathy withoutmacular edema

E11.351 Type 2 diabetes mellitus with proliferative diabetic retinopathy with macular edema

E11.359 Type 2 diabetes mellitus with proliferative diabetic retinopathy without macular edema

E11.36 Type 2 diabetes mellitus with diabetic cataract

E11.39 Type 2 diabetes mellitus with other diabetic ophthalmic complication

E11.40 Type 2 diabetes mellitus with diabetic neuropathy, unspecified

E11.41 Type 2 diabetes mellitus with diabetic mononeuropathy

E11.42 Type 2 diabetes mellitus with diabetic polyneuropathy

E11.43 Type 2 diabetes mellitus with diabetic autonomic (poly)neuropathy

E11.44 Type 2 diabetes mellitus with diabetic amyotrophy

E11.49 Type 2 diabetes mellitus with other diabetic neurological complication

E11.51 Type 2 diabetes mellitus with diabetic peripheral angiopathy without gangrene

E11.52 Type 2 diabetes mellitus with diabetic peripheral angiopathy with gangrene

E11.59 Type 2 diabetes mellitus with other circulatory complications

E11.610 Type 2 diabetes mellitus with diabetic neuropathic arthropathy

E11.618 Type 2 diabetes mellitus with other diabetic arthropathy

E11.620 Type 2 diabetes mellitus with diabetic dermatitis

15

E11.621 Type 2 diabetes mellitus with foot ulcer

E11.622 Type 2 diabetes mellitus with other skin ulcer

E11.628 Type 2 diabetes mellitus with other skin complications

E11.630 Type 2 diabetes mellitus with periodontal disease

E11.638 Type 2 diabetes mellitus with other oral complications

E11.641 Type 2 diabetes mellitus with hypoglycemia with coma

E11.649 Type 2 diabetes mellitus with hypoglycemia without coma

E11.65 Type 2 diabetes mellitus with hyperglycemia

E11.69 Type 2 diabetes mellitus with other specified complication

E11.8 Type 2 diabetes mellitus with unspecified complications

E11.9 Type 2 diabetes mellitus without complications

E13.00Other specified diabetes mellitus with hyperosmolarity without nonketotichyperglycemic-hyperosmolar coma (NKHHC)

E13.01 Other specified diabetes mellitus with hyperosmolarity with coma

E13.10 Other specified diabetes mellitus with ketoacidosis without coma

E13.11 Other specified diabetes mellitus with ketoacidosis with coma

E13.21 Other specified diabetes mellitus with diabetic nephropathy

E13.22 Other specified diabetes mellitus with diabetic chronic kidney disease

E13.29 Other specified diabetes mellitus with other diabetic kidney complication

E13.311Other specified diabetes mellitus with unspecified diabetic retinopathy with macularedema

E13.319Other specified diabetes mellitus with unspecified diabetic retinopathy without macularedema

E13.321Other specified diabetes mellitus with mild nonproliferative diabetic retinopathy withmacular edema

E13.329Other specified diabetes mellitus with mild nonproliferative diabetic retinopathy withoutmacular edema

E13.331Other specified diabetes mellitus with moderate nonproliferative diabetic retinopathywith macular edema

E13.339Other specified diabetes mellitus with moderate nonproliferative diabetic retinopathywithout macular edema

E13.341Other specified diabetes mellitus with severe nonproliferative diabetic retinopathy withmacular edema

E13.349Other specified diabetes mellitus with severe nonproliferative diabetic retinopathywithout macular edema

E13.351Other specified diabetes mellitus with proliferative diabetic retinopathy with macularedema

E13.359Other specified diabetes mellitus with proliferative diabetic retinopathy without macularedema

E13.36 Other specified diabetes mellitus with diabetic cataract

E13.39 Other specified diabetes mellitus with other diabetic ophthalmic complication

E13.40 Other specified diabetes mellitus with diabetic neuropathy, unspecified

E13.41 Other specified diabetes mellitus with diabetic mononeuropathy

E13.42 Other specified diabetes mellitus with diabetic polyneuropathy

E13.43 Other specified diabetes mellitus with diabetic autonomic (poly)neuropathy

E13.44 Other specified diabetes mellitus with diabetic amyotrophy

E13.49 Other specified diabetes mellitus with other diabetic neurological complication

E13.51 Other specified diabetes mellitus with diabetic peripheral angiopathy without gangrene

E13.52 Other specified diabetes mellitus with diabetic peripheral angiopathy with gangrene

E13.59 Other specified diabetes mellitus with other circulatory complications

E13.610 Other specified diabetes mellitus with diabetic neuropathic arthropathy

E13.618 Other specified diabetes mellitus with other diabetic arthropathy

16

E13.620 Other specified diabetes mellitus with diabetic dermatitis

E13.621 Other specified diabetes mellitus with foot ulcer

E13.622 Other specified diabetes mellitus with other skin ulcer

E13.628 Other specified diabetes mellitus with other skin complications

E13.630 Other specified diabetes mellitus with periodontal disease

E13.638 Other specified diabetes mellitus with other oral complications

E13.641 Other specified diabetes mellitus with hypoglycemia with coma

E13.649 Other specified diabetes mellitus with hypoglycemia without coma

E13.65 Other specified diabetes mellitus with hyperglycemia

E13.69 Other specified diabetes mellitus with other specified complication

E13.8 Other specified diabetes mellitus with unspecified complications

E13.9 Other specified diabetes mellitus without complications

E71.50 Peroxisomal disorder, unspecified

E71.510 Zellweger syndrome

E71.511 Neonatal adrenoleukodystrophy

E71.518 Other disorders of peroxisome biogenesis

E71.520 Childhood cerebral X-linked adrenoleukodystrophy

E71.521 Adolescent X-linked adrenoleukodystrophy

E71.522 Adrenomyeloneuropathy

E71.528 Other X-linked adrenoleukodystrophy

E71.529 X-linked adrenoleukodystrophy, unspecified type

E71.53 Other group 2 peroxisomal disorders

E71.540 Rhizomelic chondrodysplasia punctata

E71.541 Zellweger-like syndrome

E71.542 Other group 3 peroxisomal disorders

E71.548 Other peroxisomal disorders

E84.0 Cystic fibrosis with pulmonary manifestations

E84.11 Meconium ileus in cystic fibrosis

E84.19 Cystic fibrosis with other intestinal manifestations

E84.8 Cystic fibrosis with other manifestations

E84.9 Cystic fibrosis, unspecified

G12.1 Other inherited spinal muscular atrophy

G12.21 Amyotrophic lateral sclerosis

G12.8 Other spinal muscular atrophies and related syndromes

G12.9 Spinal muscular atrophy, unspecified

G25.82 Stiff-man syndrome

G35 Multiple sclerosis

G40.001Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes withseizures of localized onset, not intractable, with status epilepticus

G40.009Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes withseizures of localized onset, not intractable, without status epilepticus

G40.011Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes withseizures of localized onset, intractable, with status epilepticus

G40.019Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes withseizures of localized onset, intractable, without status epilepticus

G40.101Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes withsimple partial seizures, not intractable, with status epilepticus

G40.109Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes withsimple partial seizures, not intractable, without status epilepticus

G40.111Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes withsimple partial seizures, intractable, with status epilepticus

G40.119 Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with

17

simple partial seizures, intractable, without status epilepticus

G40.201Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes withcomplex partial seizures, not intractable, with status epilepticus

G40.209Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes withcomplex partial seizures, not intractable, without status epilepticus

G40.211Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes withcomplex partial seizures, intractable, with status epilepticus

G40.219Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes withcomplex partial seizures, intractable, without status epilepticus

G40.301Generalized idiopathic epilepsy and epileptic syndromes, not intractable, with statusepilepticus

G40.309Generalized idiopathic epilepsy and epileptic syndromes, not intractable, without statusepilepticus

G40.311Generalized idiopathic epilepsy and epileptic syndromes, intractable, with statusepilepticus

G40.319Generalized idiopathic epilepsy and epileptic syndromes, intractable, without statusepilepticus

G40.401Other generalized epilepsy and epileptic syndromes, not intractable, with statusepilepticus

G40.409Other generalized epilepsy and epileptic syndromes, not intractable, without statusepilepticus

G40.411 Other generalized epilepsy and epileptic syndromes, intractable, with status epilepticus

G40.419Other generalized epilepsy and epileptic syndromes, intractable, without statusepilepticus

G40.501 Epileptic seizures related to external causes, not intractable, with status epilepticus

G40.509 Epileptic seizures related to external causes, not intractable, without status epilepticus

G40.801 Other epilepsy, not intractable, with status epilepticus

G40.802 Other epilepsy, not intractable, without status epilepticus

G40.803 Other epilepsy, intractable, with status epilepticus

G40.804 Other epilepsy, intractable, without status epilepticus

G40.811 Lennox-Gastaut syndrome, not intractable, with status epilepticus

G40.812 Lennox-Gastaut syndrome, not intractable, without status epilepticus

G40.813 Lennox-Gastaut syndrome, intractable, with status epilepticus

G40.814 Lennox-Gastaut syndrome, intractable, without status epilepticus

G40.821 Epileptic spasms, not intractable, with status epilepticus

G40.822 Epileptic spasms, not intractable, without status epilepticus

G40.823 Epileptic spasms, intractable, with status epilepticus

G40.824 Epileptic spasms, intractable, without status epilepticus

G40.89 Other seizures

G40.901 Epilepsy, unspecified, not intractable, with status epilepticus

G40.909 Epilepsy, unspecified, not intractable, without status epilepticus

G40.911 Epilepsy, unspecified, intractable, with status epilepticus

G40.919 Epilepsy, unspecified, intractable, without status epilepticus

G40.A01 Absence epileptic syndrome, not intractable, with status epilepticus

G40.A09 Absence epileptic syndrome, not intractable, without status epilepticus

G40.A11 Absence epileptic syndrome, intractable, with status epilepticus

G40.A19 Absence epileptic syndrome, intractable, without status epilepticus

G40.B01 Juvenile myoclonic epilepsy, not intractable, with status epilepticus

G40.B09 Juvenile myoclonic epilepsy, not intractable, without status epilepticus

G40.B11 Juvenile myoclonic epilepsy, intractable, with status epilepticus

G40.B19 Juvenile myoclonic epilepsy, intractable, without status epilepticus

G60.0 Hereditary motor and sensory neuropathy

18

G60.1 Refsum's disease

G60.3 Idiopathic progressive neuropathy

G60.8 Other hereditary and idiopathic neuropathies

G60.9 Hereditary and idiopathic neuropathy, unspecified

G61.0 Guillain-Barre syndrome

G61.81 Chronic inflammatory demyelinating polyneuritis

G62.81 Critical illness polyneuropathy

G70.00 Myasthenia gravis without (acute) exacerbation

G70.01 Myasthenia gravis with (acute) exacerbation

G70.80 Lambert-Eaton syndrome, unspecified

G70.81 Lambert-Eaton syndrome in disease classified elsewhere

G73.1 Lambert-Eaton syndrome in neoplastic disease

G90.01 Carotid sinus syncope

G90.09 Other idiopathic peripheral autonomic neuropathy

H20.9 Unspecified iridocyclitis

H46.9 Unspecified optic neuritis

H66.004Acute suppurative otitis media without spontaneous rupture of ear drum, recurrent, rightear

H66.005Acute suppurative otitis media without spontaneous rupture of ear drum, recurrent, leftear

H66.006Acute suppurative otitis media without spontaneous rupture of ear drum, recurrent,bilateral

H66.007Acute suppurative otitis media without spontaneous rupture of ear drum, recurrent,unspecified ear

H66.014Acute suppurative otitis media with spontaneous rupture of ear drum, recurrent, rightear

H66.015 Acute suppurative otitis media with spontaneous rupture of ear drum, recurrent, left ear

H66.016Acute suppurative otitis media with spontaneous rupture of ear drum, recurrent,bilateral

H66.017Acute suppurative otitis media with spontaneous rupture of ear drum, recurrent,unspecified ear

I42.0 Dilated cardiomyopathy

I42.5 Other restrictive cardiomyopathy

I42.8 Other cardiomyopathies

I42.9 Cardiomyopathy, unspecified

I77.6 Arteritis, unspecified

J18.8 Other pneumonia, unspecified organism

J18.9 Pneumonia, unspecified organism

J32.0 Chronic maxillary sinusitis

J32.1 Chronic frontal sinusitis

J32.2 Chronic ethmoidal sinusitis

J32.3 Chronic sphenoidal sinusitis

J32.4 Chronic pansinusitis

J32.8 Other chronic sinusitis

J32.9 Chronic sinusitis, unspecified

J44.0 Chronic obstructive pulmonary disease with acute lower respiratory infection

J44.1 Chronic obstructive pulmonary disease with (acute) exacerbation

J44.9 Chronic obstructive pulmonary disease, unspecified

J45.20 Mild intermittent asthma, uncomplicated

J45.21 Mild intermittent asthma with (acute) exacerbation

J45.22 Mild intermittent asthma with status asthmaticus

J45.30 Mild persistent asthma, uncomplicated

19

J45.31 Mild persistent asthma with (acute) exacerbation

J45.32 Mild persistent asthma with status asthmaticus

J45.40 Moderate persistent asthma, uncomplicated

J45.41 Moderate persistent asthma with (acute) exacerbation

J45.42 Moderate persistent asthma with status asthmaticus

J45.50 Severe persistent asthma, uncomplicated

J45.51 Severe persistent asthma with (acute) exacerbation

J45.52 Severe persistent asthma with status asthmaticus

J45.901 Unspecified asthma with (acute) exacerbation

J45.902 Unspecified asthma with status asthmaticus

J45.909 Unspecified asthma, uncomplicated

J45.990 Exercise induced bronchospasm

J45.991 Cough variant asthma

J45.998 Other asthma

K21.9 Gastro-esophageal reflux disease without esophagitis

L10.0 Pemphigus vulgaris

L10.1 Pemphigus vegetans

L10.2 Pemphigus foliaceous

L10.3 Brazilian pemphigus [fogo selvagem]

L10.4 Pemphigus erythematosus

L10.5 Drug-induced pemphigus

L10.81 Paraneoplastic pemphigus

L10.89 Other pemphigus

L10.9 Pemphigus, unspecified

L12.0 Bullous pemphigoid

L12.8 Other pemphigoid

L12.9 Pemphigoid, unspecified

M05.00 Felty's syndrome, unspecified site

M05.011 Felty's syndrome, right shoulder

M05.012 Felty's syndrome, left shoulder

M05.019 Felty's syndrome, unspecified shoulder

M05.021 Felty's syndrome, right elbow

M05.022 Felty's syndrome, left elbow

M05.029 Felty's syndrome, unspecified elbow

M05.031 Felty's syndrome, right wrist

M05.032 Felty's syndrome, left wrist

M05.039 Felty's syndrome, unspecified wrist

M05.041 Felty's syndrome, right hand

M05.042 Felty's syndrome, left hand

M05.049 Felty's syndrome, unspecified hand

M05.051 Felty's syndrome, right hip

M05.052 Felty's syndrome, left hip

M05.059 Felty's syndrome, unspecified hip

M05.061 Felty's syndrome, right knee

M05.062 Felty's syndrome, left knee

M05.069 Felty's syndrome, unspecified knee

M05.071 Felty's syndrome, right ankle and foot

M05.072 Felty's syndrome, left ankle and foot

M05.079 Felty's syndrome, unspecified ankle and foot

M05.09 Felty's syndrome, multiple sites

M05.20 Rheumatoid vasculitis with rheumatoid arthritis of unspecified site

20

M05.211 Rheumatoid vasculitis with rheumatoid arthritis of right shoulder

M05.212 Rheumatoid vasculitis with rheumatoid arthritis of left shoulder

M05.219 Rheumatoid vasculitis with rheumatoid arthritis of unspecified shoulder

M05.221 Rheumatoid vasculitis with rheumatoid arthritis of right elbow

M05.222 Rheumatoid vasculitis with rheumatoid arthritis of left elbow

M05.229 Rheumatoid vasculitis with rheumatoid arthritis of unspecified elbow

M05.231 Rheumatoid vasculitis with rheumatoid arthritis of right wrist

M05.232 Rheumatoid vasculitis with rheumatoid arthritis of left wrist

M05.239 Rheumatoid vasculitis with rheumatoid arthritis of unspecified wrist

M05.241 Rheumatoid vasculitis with rheumatoid arthritis of right hand

M05.242 Rheumatoid vasculitis with rheumatoid arthritis of left hand

M05.249 Rheumatoid vasculitis with rheumatoid arthritis of unspecified hand

M05.251 Rheumatoid vasculitis with rheumatoid arthritis of right hip

M05.252 Rheumatoid vasculitis with rheumatoid arthritis of left hip

M05.259 Rheumatoid vasculitis with rheumatoid arthritis of unspecified hip

M05.261 Rheumatoid vasculitis with rheumatoid arthritis of right knee

M05.262 Rheumatoid vasculitis with rheumatoid arthritis of left knee

M05.269 Rheumatoid vasculitis with rheumatoid arthritis of unspecified knee

M05.271 Rheumatoid vasculitis with rheumatoid arthritis of right ankle and foot

M05.272 Rheumatoid vasculitis with rheumatoid arthritis of left ankle and foot

M05.279 Rheumatoid vasculitis with rheumatoid arthritis of unspecified ankle and foot

M05.29 Rheumatoid vasculitis with rheumatoid arthritis of multiple sites

M05.30 Rheumatoid heart disease with rheumatoid arthritis of unspecified site

M05.311 Rheumatoid heart disease with rheumatoid arthritis of right shoulder

M05.312 Rheumatoid heart disease with rheumatoid arthritis of left shoulder

M05.319 Rheumatoid heart disease with rheumatoid arthritis of unspecified shoulder

M05.321 Rheumatoid heart disease with rheumatoid arthritis of right elbow

M05.322 Rheumatoid heart disease with rheumatoid arthritis of left elbow

M05.329 Rheumatoid heart disease with rheumatoid arthritis of unspecified elbow

M05.331 Rheumatoid heart disease with rheumatoid arthritis of right wrist

M05.332 Rheumatoid heart disease with rheumatoid arthritis of left wrist

M05.339 Rheumatoid heart disease with rheumatoid arthritis of unspecified wrist

M05.341 Rheumatoid heart disease with rheumatoid arthritis of right hand

M05.342 Rheumatoid heart disease with rheumatoid arthritis of left hand

M05.349 Rheumatoid heart disease with rheumatoid arthritis of unspecified hand

M05.351 Rheumatoid heart disease with rheumatoid arthritis of right hip

M05.352 Rheumatoid heart disease with rheumatoid arthritis of left hip

M05.359 Rheumatoid heart disease with rheumatoid arthritis of unspecified hip

M05.361 Rheumatoid heart disease with rheumatoid arthritis of right knee

M05.362 Rheumatoid heart disease with rheumatoid arthritis of left knee

M05.369 Rheumatoid heart disease with rheumatoid arthritis of unspecified knee

M05.371 Rheumatoid heart disease with rheumatoid arthritis of right ankle and foot

M05.372 Rheumatoid heart disease with rheumatoid arthritis of left ankle and foot

M05.379 Rheumatoid heart disease with rheumatoid arthritis of unspecified ankle and foot

M05.39 Rheumatoid heart disease with rheumatoid arthritis of multiple sites

M05.40 Rheumatoid myopathy with rheumatoid arthritis of unspecified site

M05.411 Rheumatoid myopathy with rheumatoid arthritis of right shoulder

M05.412 Rheumatoid myopathy with rheumatoid arthritis of left shoulder

M05.419 Rheumatoid myopathy with rheumatoid arthritis of unspecified shoulder

M05.421 Rheumatoid myopathy with rheumatoid arthritis of right elbow

M05.422 Rheumatoid myopathy with rheumatoid arthritis of left elbow

21

M05.429 Rheumatoid myopathy with rheumatoid arthritis of unspecified elbow

M05.431 Rheumatoid myopathy with rheumatoid arthritis of right wrist

M05.432 Rheumatoid myopathy with rheumatoid arthritis of left wrist

M05.439 Rheumatoid myopathy with rheumatoid arthritis of unspecified wrist

M05.441 Rheumatoid myopathy with rheumatoid arthritis of right hand

M05.442 Rheumatoid myopathy with rheumatoid arthritis of left hand

M05.449 Rheumatoid myopathy with rheumatoid arthritis of unspecified hand

M05.451 Rheumatoid myopathy with rheumatoid arthritis of right hip

M05.452 Rheumatoid myopathy with rheumatoid arthritis of left hip

M05.459 Rheumatoid myopathy with rheumatoid arthritis of unspecified hip

M05.461 Rheumatoid myopathy with rheumatoid arthritis of right knee

M05.462 Rheumatoid myopathy with rheumatoid arthritis of left knee

M05.469 Rheumatoid myopathy with rheumatoid arthritis of unspecified knee

M05.471 Rheumatoid myopathy with rheumatoid arthritis of right ankle and foot

M05.472 Rheumatoid myopathy with rheumatoid arthritis of left ankle and foot

M05.479 Rheumatoid myopathy with rheumatoid arthritis of unspecified ankle and foot

M05.49 Rheumatoid myopathy with rheumatoid arthritis of multiple sites

M05.50 Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified site

M05.511 Rheumatoid polyneuropathy with rheumatoid arthritis of right shoulder

M05.512 Rheumatoid polyneuropathy with rheumatoid arthritis of left shoulder

M05.519 Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified shoulder

M05.521 Rheumatoid polyneuropathy with rheumatoid arthritis of right elbow

M05.522 Rheumatoid polyneuropathy with rheumatoid arthritis of left elbow

M05.529 Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified elbow

M05.531 Rheumatoid polyneuropathy with rheumatoid arthritis of right wrist

M05.532 Rheumatoid polyneuropathy with rheumatoid arthritis of left wrist

M05.539 Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified wrist

M05.541 Rheumatoid polyneuropathy with rheumatoid arthritis of right hand

M05.542 Rheumatoid polyneuropathy with rheumatoid arthritis of left hand

M05.549 Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified hand

M05.551 Rheumatoid polyneuropathy with rheumatoid arthritis of right hip

M05.552 Rheumatoid polyneuropathy with rheumatoid arthritis of left hip

M05.559 Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified hip

M05.561 Rheumatoid polyneuropathy with rheumatoid arthritis of right knee

M05.562 Rheumatoid polyneuropathy with rheumatoid arthritis of left knee

M05.569 Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified knee

M05.571 Rheumatoid polyneuropathy with rheumatoid arthritis of right ankle and foot

M05.572 Rheumatoid polyneuropathy with rheumatoid arthritis of left ankle and foot

M05.579 Rheumatoid polyneuropathy with rheumatoid arthritis of unspecified ankle and foot

M05.59 Rheumatoid polyneuropathy with rheumatoid arthritis of multiple sites

M05.60 Rheumatoid arthritis of unspecified site with involvement of other organs and systems

M05.611 Rheumatoid arthritis of right shoulder with involvement of other organs and systems

M05.612 Rheumatoid arthritis of left shoulder with involvement of other organs and systems

M05.619Rheumatoid arthritis of unspecified shoulder with involvement of other organs andsystems

M05.621 Rheumatoid arthritis of right elbow with involvement of other organs and systems

M05.622 Rheumatoid arthritis of left elbow with involvement of other organs and systems

M05.629Rheumatoid arthritis of unspecified elbow with involvement of other organs andsystems

M05.631 Rheumatoid arthritis of right wrist with involvement of other organs and systems

M05.632 Rheumatoid arthritis of left wrist with involvement of other organs and systems

22

M05.639 Rheumatoid arthritis of unspecified wrist with involvement of other organs and systems

M05.641 Rheumatoid arthritis of right hand with involvement of other organs and systems

M05.642 Rheumatoid arthritis of left hand with involvement of other organs and systems

M05.649 Rheumatoid arthritis of unspecified hand with involvement of other organs and systems

M05.651 Rheumatoid arthritis of right hip with involvement of other organs and systems

M05.652 Rheumatoid arthritis of left hip with involvement of other organs and systems

M05.659 Rheumatoid arthritis of unspecified hip with involvement of other organs and systems

M05.661 Rheumatoid arthritis of right knee with involvement of other organs and systems

M05.662 Rheumatoid arthritis of left knee with involvement of other organs and systems

M05.669 Rheumatoid arthritis of unspecified knee with involvement of other organs and systems

M05.671Rheumatoid arthritis of right ankle and foot with involvement of other organs andsystems

M05.672Rheumatoid arthritis of left ankle and foot with involvement of other organs andsystems

M05.679Rheumatoid arthritis of unspecified ankle and foot with involvement of other organs andsystems

M05.69 Rheumatoid arthritis of multiple sites with involvement of other organs and systems

M05.70Rheumatoid arthritis with rheumatoid factor of unspecified site without organ or systemsinvolvement

M05.711Rheumatoid arthritis with rheumatoid factor of right shoulder without organ or systemsinvolvement

M05.712Rheumatoid arthritis with rheumatoid factor of left shoulder without organ or systemsinvolvement

M05.719Rheumatoid arthritis with rheumatoid factor of unspecified shoulder without organ orsystems involvement

M05.721Rheumatoid arthritis with rheumatoid factor of right elbow without organ or systemsinvolvement

M05.722Rheumatoid arthritis with rheumatoid factor of left elbow without organ or systemsinvolvement

M05.729Rheumatoid arthritis with rheumatoid factor of unspecified elbow without organ orsystems involvement

M05.731Rheumatoid arthritis with rheumatoid factor of right wrist without organ or systemsinvolvement

M05.732Rheumatoid arthritis with rheumatoid factor of left wrist without organ or systemsinvolvement

M05.739Rheumatoid arthritis with rheumatoid factor of unspecified wrist without organ orsystems involvement

M05.741Rheumatoid arthritis with rheumatoid factor of right hand without organ or systemsinvolvement

M05.742Rheumatoid arthritis with rheumatoid factor of left hand without organ or systemsinvolvement

M05.749Rheumatoid arthritis with rheumatoid factor of unspecified hand without organ orsystems involvement

M05.751Rheumatoid arthritis with rheumatoid factor of right hip without organ or systemsinvolvement

M05.752Rheumatoid arthritis with rheumatoid factor of left hip without organ or systemsinvolvement

M05.759Rheumatoid arthritis with rheumatoid factor of unspecified hip without organ or systemsinvolvement

M05.761Rheumatoid arthritis with rheumatoid factor of right knee without organ or systemsinvolvement

M05.762Rheumatoid arthritis with rheumatoid factor of left knee without organ or systemsinvolvement

23

M05.769Rheumatoid arthritis with rheumatoid factor of unspecified knee without organ orsystems involvement

M05.771Rheumatoid arthritis with rheumatoid factor of right ankle and foot without organ orsystems involvement

M05.772Rheumatoid arthritis with rheumatoid factor of left ankle and foot without organ orsystems involvement

M05.779Rheumatoid arthritis with rheumatoid factor of unspecified ankle and foot without organor systems involvement

M05.79Rheumatoid arthritis with rheumatoid factor of multiple sites without organ or systemsinvolvement

M05.80 Other rheumatoid arthritis with rheumatoid factor of unspecified site

M05.811 Other rheumatoid arthritis with rheumatoid factor of right shoulder

M05.812 Other rheumatoid arthritis with rheumatoid factor of left shoulder

M05.819 Other rheumatoid arthritis with rheumatoid factor of unspecified shoulder

M05.821 Other rheumatoid arthritis with rheumatoid factor of right elbow

M05.822 Other rheumatoid arthritis with rheumatoid factor of left elbow

M05.829 Other rheumatoid arthritis with rheumatoid factor of unspecified elbow

M05.831 Other rheumatoid arthritis with rheumatoid factor of right wrist

M05.832 Other rheumatoid arthritis with rheumatoid factor of left wrist

M05.839 Other rheumatoid arthritis with rheumatoid factor of unspecified wrist

M05.841 Other rheumatoid arthritis with rheumatoid factor of right hand

M05.842 Other rheumatoid arthritis with rheumatoid factor of left hand

M05.849 Other rheumatoid arthritis with rheumatoid factor of unspecified hand

M05.851 Other rheumatoid arthritis with rheumatoid factor of right hip

M05.852 Other rheumatoid arthritis with rheumatoid factor of left hip

M05.859 Other rheumatoid arthritis with rheumatoid factor of unspecified hip

M05.861 Other rheumatoid arthritis with rheumatoid factor of right knee

M05.862 Other rheumatoid arthritis with rheumatoid factor of left knee

M05.869 Other rheumatoid arthritis with rheumatoid factor of unspecified knee

M05.871 Other rheumatoid arthritis with rheumatoid factor of right ankle and foot

M05.872 Other rheumatoid arthritis with rheumatoid factor of left ankle and foot

M05.879 Other rheumatoid arthritis with rheumatoid factor of unspecified ankle and foot

M05.89 Other rheumatoid arthritis with rheumatoid factor of multiple sites

M05.9 Rheumatoid arthritis with rheumatoid factor, unspecified

M06.00 Rheumatoid arthritis without rheumatoid factor, unspecified site

M06.011 Rheumatoid arthritis without rheumatoid factor, right shoulder

M06.012 Rheumatoid arthritis without rheumatoid factor, left shoulder

M06.019 Rheumatoid arthritis without rheumatoid factor, unspecified shoulder

M06.021 Rheumatoid arthritis without rheumatoid factor, right elbow

M06.022 Rheumatoid arthritis without rheumatoid factor, left elbow

M06.029 Rheumatoid arthritis without rheumatoid factor, unspecified elbow

M06.031 Rheumatoid arthritis without rheumatoid factor, right wrist

M06.032 Rheumatoid arthritis without rheumatoid factor, left wrist

M06.039 Rheumatoid arthritis without rheumatoid factor, unspecified wrist

M06.041 Rheumatoid arthritis without rheumatoid factor, right hand

M06.042 Rheumatoid arthritis without rheumatoid factor, left hand

M06.049 Rheumatoid arthritis without rheumatoid factor, unspecified hand

M06.051 Rheumatoid arthritis without rheumatoid factor, right hip

M06.052 Rheumatoid arthritis without rheumatoid factor, left hip

M06.059 Rheumatoid arthritis without rheumatoid factor, unspecified hip

M06.061 Rheumatoid arthritis without rheumatoid factor, right knee

24

M06.062 Rheumatoid arthritis without rheumatoid factor, left knee

M06.069 Rheumatoid arthritis without rheumatoid factor, unspecified knee

M06.071 Rheumatoid arthritis without rheumatoid factor, right ankle and foot

M06.072 Rheumatoid arthritis without rheumatoid factor, left ankle and foot

M06.079 Rheumatoid arthritis without rheumatoid factor, unspecified ankle and foot

M06.08 Rheumatoid arthritis without rheumatoid factor, vertebrae

M06.09 Rheumatoid arthritis without rheumatoid factor, multiple sites

M06.1 Adult-onset Still's disease

M06.20 Rheumatoid bursitis, unspecified site

M06.211 Rheumatoid bursitis, right shoulder

M06.212 Rheumatoid bursitis, left shoulder

M06.219 Rheumatoid bursitis, unspecified shoulder

M06.221 Rheumatoid bursitis, right elbow

M06.222 Rheumatoid bursitis, left elbow

M06.229 Rheumatoid bursitis, unspecified elbow

M06.231 Rheumatoid bursitis, right wrist

M06.232 Rheumatoid bursitis, left wrist

M06.239 Rheumatoid bursitis, unspecified wrist

M06.241 Rheumatoid bursitis, right hand

M06.242 Rheumatoid bursitis, left hand

M06.249 Rheumatoid bursitis, unspecified hand

M06.251 Rheumatoid bursitis, right hip

M06.252 Rheumatoid bursitis, left hip

M06.259 Rheumatoid bursitis, unspecified hip

M06.261 Rheumatoid bursitis, right knee

M06.262 Rheumatoid bursitis, left knee

M06.269 Rheumatoid bursitis, unspecified knee

M06.271 Rheumatoid bursitis, right ankle and foot

M06.272 Rheumatoid bursitis, left ankle and foot

M06.279 Rheumatoid bursitis, unspecified ankle and foot

M06.28 Rheumatoid bursitis, vertebrae

M06.29 Rheumatoid bursitis, multiple sites

M06.30 Rheumatoid nodule, unspecified site

M06.311 Rheumatoid nodule, right shoulder

M06.312 Rheumatoid nodule, left shoulder

M06.319 Rheumatoid nodule, unspecified shoulder

M06.321 Rheumatoid nodule, right elbow

M06.322 Rheumatoid nodule, left elbow

M06.329 Rheumatoid nodule, unspecified elbow

M06.331 Rheumatoid nodule, right wrist

M06.332 Rheumatoid nodule, left wrist

M06.339 Rheumatoid nodule, unspecified wrist

M06.341 Rheumatoid nodule, right hand

M06.342 Rheumatoid nodule, left hand

M06.349 Rheumatoid nodule, unspecified hand

M06.351 Rheumatoid nodule, right hip

M06.352 Rheumatoid nodule, left hip

M06.359 Rheumatoid nodule, unspecified hip

M06.361 Rheumatoid nodule, right knee

M06.362 Rheumatoid nodule, left knee

M06.369 Rheumatoid nodule, unspecified knee

25

M06.371 Rheumatoid nodule, right ankle and foot

M06.372 Rheumatoid nodule, left ankle and foot

M06.379 Rheumatoid nodule, unspecified ankle and foot

M06.38 Rheumatoid nodule, vertebrae

M06.39 Rheumatoid nodule, multiple sites

M06.80 Other specified rheumatoid arthritis, unspecified site

M06.811 Other specified rheumatoid arthritis, right shoulder

M06.812 Other specified rheumatoid arthritis, left shoulder

M06.819 Other specified rheumatoid arthritis, unspecified shoulder

M06.821 Other specified rheumatoid arthritis, right elbow

M06.822 Other specified rheumatoid arthritis, left elbow

M06.829 Other specified rheumatoid arthritis, unspecified elbow

M06.831 Other specified rheumatoid arthritis, right wrist

M06.832 Other specified rheumatoid arthritis, left wrist

M06.839 Other specified rheumatoid arthritis, unspecified wrist

M06.841 Other specified rheumatoid arthritis, right hand

M06.842 Other specified rheumatoid arthritis, left hand

M06.849 Other specified rheumatoid arthritis, unspecified hand

M06.851 Other specified rheumatoid arthritis, right hip

M06.852 Other specified rheumatoid arthritis, left hip

M06.859 Other specified rheumatoid arthritis, unspecified hip

M06.861 Other specified rheumatoid arthritis, right knee

M06.862 Other specified rheumatoid arthritis, left knee

M06.869 Other specified rheumatoid arthritis, unspecified knee

M06.871 Other specified rheumatoid arthritis, right ankle and foot

M06.872 Other specified rheumatoid arthritis, left ankle and foot

M06.879 Other specified rheumatoid arthritis, unspecified ankle and foot

M06.88 Other specified rheumatoid arthritis, vertebrae

M06.89 Other specified rheumatoid arthritis, multiple sites

M06.9 Rheumatoid arthritis, unspecified

M08.00 Unspecified juvenile rheumatoid arthritis of unspecified site

M08.011 Unspecified juvenile rheumatoid arthritis, right shoulder

M08.012 Unspecified juvenile rheumatoid arthritis, left shoulder

M08.019 Unspecified juvenile rheumatoid arthritis, unspecified shoulder

M08.021 Unspecified juvenile rheumatoid arthritis, right elbow

M08.022 Unspecified juvenile rheumatoid arthritis, left elbow

M08.029 Unspecified juvenile rheumatoid arthritis, unspecified elbow

M08.031 Unspecified juvenile rheumatoid arthritis, right wrist

M08.032 Unspecified juvenile rheumatoid arthritis, left wrist

M08.039 Unspecified juvenile rheumatoid arthritis, unspecified wrist

M08.041 Unspecified juvenile rheumatoid arthritis, right hand

M08.042 Unspecified juvenile rheumatoid arthritis, left hand

M08.049 Unspecified juvenile rheumatoid arthritis, unspecified hand

M08.051 Unspecified juvenile rheumatoid arthritis, right hip

M08.052 Unspecified juvenile rheumatoid arthritis, left hip

M08.059 Unspecified juvenile rheumatoid arthritis, unspecified hip

M08.061 Unspecified juvenile rheumatoid arthritis, right knee

M08.062 Unspecified juvenile rheumatoid arthritis, left knee

M08.069 Unspecified juvenile rheumatoid arthritis, unspecified knee

M08.071 Unspecified juvenile rheumatoid arthritis, right ankle and foot

M08.072 Unspecified juvenile rheumatoid arthritis, left ankle and foot

26

M08.079 Unspecified juvenile rheumatoid arthritis, unspecified ankle and foot

M08.08 Unspecified juvenile rheumatoid arthritis, vertebrae

M08.09 Unspecified juvenile rheumatoid arthritis, multiple sites

M08.20 Juvenile rheumatoid arthritis with systemic onset, unspecified site

M08.211 Juvenile rheumatoid arthritis with systemic onset, right shoulder

M08.212 Juvenile rheumatoid arthritis with systemic onset, left shoulder

M08.219 Juvenile rheumatoid arthritis with systemic onset, unspecified shoulder

M08.221 Juvenile rheumatoid arthritis with systemic onset, right elbow

M08.222 Juvenile rheumatoid arthritis with systemic onset, left elbow

M08.229 Juvenile rheumatoid arthritis with systemic onset, unspecified elbow

M08.231 Juvenile rheumatoid arthritis with systemic onset, right wrist

M08.232 Juvenile rheumatoid arthritis with systemic onset, left wrist

M08.239 Juvenile rheumatoid arthritis with systemic onset, unspecified wrist

M08.241 Juvenile rheumatoid arthritis with systemic onset, right hand

M08.242 Juvenile rheumatoid arthritis with systemic onset, left hand

M08.249 Juvenile rheumatoid arthritis with systemic onset, unspecified hand

M08.251 Juvenile rheumatoid arthritis with systemic onset, right hip

M08.252 Juvenile rheumatoid arthritis with systemic onset, left hip

M08.259 Juvenile rheumatoid arthritis with systemic onset, unspecified hip

M08.261 Juvenile rheumatoid arthritis with systemic onset, right knee

M08.262 Juvenile rheumatoid arthritis with systemic onset, left knee

M08.269 Juvenile rheumatoid arthritis with systemic onset, unspecified knee

M08.271 Juvenile rheumatoid arthritis with systemic onset, right ankle and foot

M08.272 Juvenile rheumatoid arthritis with systemic onset, left ankle and foot

M08.279 Juvenile rheumatoid arthritis with systemic onset, unspecified ankle and foot

M08.28 Juvenile rheumatoid arthritis with systemic onset, vertebrae

M08.29 Juvenile rheumatoid arthritis with systemic onset, multiple sites

M08.3 Juvenile rheumatoid polyarthritis (seronegative)

M08.40 Pauciarticular juvenile rheumatoid arthritis, unspecified site

M08.411 Pauciarticular juvenile rheumatoid arthritis, right shoulder

M08.412 Pauciarticular juvenile rheumatoid arthritis, left shoulder

M08.419 Pauciarticular juvenile rheumatoid arthritis, unspecified shoulder

M08.421 Pauciarticular juvenile rheumatoid arthritis, right elbow

M08.422 Pauciarticular juvenile rheumatoid arthritis, left elbow

M08.429 Pauciarticular juvenile rheumatoid arthritis, unspecified elbow

M08.431 Pauciarticular juvenile rheumatoid arthritis, right wrist

M08.432 Pauciarticular juvenile rheumatoid arthritis, left wrist

M08.439 Pauciarticular juvenile rheumatoid arthritis, unspecified wrist

M08.441 Pauciarticular juvenile rheumatoid arthritis, right hand

M08.442 Pauciarticular juvenile rheumatoid arthritis, left hand

M08.449 Pauciarticular juvenile rheumatoid arthritis, unspecified hand

M08.451 Pauciarticular juvenile rheumatoid arthritis, right hip

M08.452 Pauciarticular juvenile rheumatoid arthritis, left hip

M08.459 Pauciarticular juvenile rheumatoid arthritis, unspecified hip

M08.461 Pauciarticular juvenile rheumatoid arthritis, right knee

M08.462 Pauciarticular juvenile rheumatoid arthritis, left knee

M08.469 Pauciarticular juvenile rheumatoid arthritis, unspecified knee

M08.471 Pauciarticular juvenile rheumatoid arthritis, right ankle and foot

M08.472 Pauciarticular juvenile rheumatoid arthritis, left ankle and foot

M08.479 Pauciarticular juvenile rheumatoid arthritis, unspecified ankle and foot

M08.48 Pauciarticular juvenile rheumatoid arthritis, vertebrae

27

M08.80 Other juvenile arthritis, unspecified site

M08.811 Other juvenile arthritis, right shoulder

M08.812 Other juvenile arthritis, left shoulder

M08.819 Other juvenile arthritis, unspecified shoulder

M08.821 Other juvenile arthritis, right elbow

M08.822 Other juvenile arthritis, left elbow

M08.829 Other juvenile arthritis, unspecified elbow

M08.831 Other juvenile arthritis, right wrist

M08.832 Other juvenile arthritis, left wrist

M08.839 Other juvenile arthritis, unspecified wrist

M08.841 Other juvenile arthritis, right hand

M08.842 Other juvenile arthritis, left hand

M08.849 Other juvenile arthritis, unspecified hand

M08.851 Other juvenile arthritis, right hip

M08.852 Other juvenile arthritis, left hip

M08.859 Other juvenile arthritis, unspecified hip

M08.861 Other juvenile arthritis, right knee

M08.862 Other juvenile arthritis, left knee

M08.869 Other juvenile arthritis, unspecified knee

M08.871 Other juvenile arthritis, right ankle and foot

M08.872 Other juvenile arthritis, left ankle and foot

M08.879 Other juvenile arthritis, unspecified ankle and foot

M08.88 Other juvenile arthritis, other specified site

M08.89 Other juvenile arthritis, multiple sites

M08.90 Juvenile arthritis, unspecified, unspecified site

M08.911 Juvenile arthritis, unspecified, right shoulder

M08.912 Juvenile arthritis, unspecified, left shoulder

M08.919 Juvenile arthritis, unspecified, unspecified shoulder

M08.921 Juvenile arthritis, unspecified, right elbow

M08.922 Juvenile arthritis, unspecified, left elbow

M08.929 Juvenile arthritis, unspecified, unspecified elbow

M08.931 Juvenile arthritis, unspecified, right wrist

M08.932 Juvenile arthritis, unspecified, left wrist

M08.939 Juvenile arthritis, unspecified, unspecified wrist

M08.941 Juvenile arthritis, unspecified, right hand

M08.942 Juvenile arthritis, unspecified, left hand

M08.949 Juvenile arthritis, unspecified, unspecified hand

M08.951 Juvenile arthritis, unspecified, right hip

M08.952 Juvenile arthritis, unspecified, left hip

M08.959 Juvenile arthritis, unspecified, unspecified hip

M08.961 Juvenile arthritis, unspecified, right knee

M08.962 Juvenile arthritis, unspecified, left knee

M08.969 Juvenile arthritis, unspecified, unspecified knee

M08.971 Juvenile arthritis, unspecified, right ankle and foot

M08.972 Juvenile arthritis, unspecified, left ankle and foot

M08.979 Juvenile arthritis, unspecified, unspecified ankle and foot

M08.98 Juvenile arthritis, unspecified, vertebrae

M08.99 Juvenile arthritis, unspecified, multiple sites

M30.1 Polyarteritis with lung involvement [Churg-Strauss]

M30.3 Mucocutaneous lymph node syndrome [Kawasaki]

M31.0 Hypersensitivity angiitis

28

M31.1 Thrombotic microangiopathy

M31.30 Wegener's granulomatosis without renal involvement

M31.31 Wegener's granulomatosis with renal involvement

M32.0 Drug-induced systemic lupus erythematosus

M32.10 Systemic lupus erythematosus, organ or system involvement unspecified

M32.11 Endocarditis in systemic lupus erythematosus

M32.12 Pericarditis in systemic lupus erythematosus

M32.13 Lung involvement in systemic lupus erythematosus

M32.14 Glomerular disease in systemic lupus erythematosus

M32.15 Tubulo-interstitial nephropathy in systemic lupus erythematosus

M32.19 Other organ or system involvement in systemic lupus erythematosus

M32.8 Other forms of systemic lupus erythematosus

M32.9 Systemic lupus erythematosus, unspecified

M33.00 Juvenile dermatopolymyositis, organ involvement unspecified

M33.01 Juvenile dermatopolymyositis with respiratory involvement

M33.02 Juvenile dermatopolymyositis with myopathy

M33.09 Juvenile dermatopolymyositis with other organ involvement

M33.10 Other dermatopolymyositis, organ involvement unspecified

M33.11 Other dermatopolymyositis with respiratory involvement

M33.12 Other dermatopolymyositis with myopathy

M33.19 Other dermatopolymyositis with other organ involvement

M33.20 Polymyositis, organ involvement unspecified

M33.21 Polymyositis with respiratory involvement

M33.22 Polymyositis with myopathy

M33.29 Polymyositis with other organ involvement

M33.90 Dermatopolymyositis, unspecified, organ involvement unspecified

M33.91 Dermatopolymyositis, unspecified with respiratory involvement

M33.92 Dermatopolymyositis, unspecified with myopathy

M33.99 Dermatopolymyositis, unspecified with other organ involvement

M34.0 Progressive systemic sclerosis

M34.1 CR(E)ST syndrome

M34.2 Systemic sclerosis induced by drug and chemical

M34.81 Systemic sclerosis with lung involvement

M34.82 Systemic sclerosis with myopathy

M34.83 Systemic sclerosis with polyneuropathy

M34.89 Other systemic sclerosis

M34.9 Systemic sclerosis, unspecified

M35.2 Behçet's disease

M36.0 Dermato(poly)myositis in neoplastic disease

M60.80 Other myositis, unspecified site

M60.811 Other myositis, right shoulder

M60.812 Other myositis, left shoulder

M60.819 Other myositis, unspecified shoulder

M60.821 Other myositis, right upper arm

M60.822 Other myositis, left upper arm

M60.829 Other myositis, unspecified upper arm

M60.831 Other myositis, right forearm

M60.832 Other myositis, left forearm

M60.839 Other myositis, unspecified forearm

M60.841 Other myositis, right hand

M60.842 Other myositis, left hand

29

M60.849 Other myositis, unspecified hand

M60.851 Other myositis, right thigh

M60.852 Other myositis, left thigh

M60.859 Other myositis, unspecified thigh

M60.861 Other myositis, right lower leg

M60.862 Other myositis, left lower leg

M60.869 Other myositis, unspecified lower leg

M60.871 Other myositis, right ankle and foot

M60.872 Other myositis, left ankle and foot

M60.879 Other myositis, unspecified ankle and foot

M60.88 Other myositis, other site

M60.89 Other myositis, multiple sites

M60.9 Myositis, unspecified

M79.1 Myalgia

N96 Recurrent pregnancy loss

O26.20 Pregnancy care for patient with recurrent pregnancy loss, unspecified trimester

O26.21 Pregnancy care for patient with recurrent pregnancy loss, first trimester

O26.22 Pregnancy care for patient with recurrent pregnancy loss, second trimester

O26.23 Pregnancy care for patient with recurrent pregnancy loss, third trimester

Q81.0 Epidermolysis bullosa simplex

Q81.1 Epidermolysis bullosa letalis

Q81.2 Epidermolysis bullosa dystrophica

Q81.8 Other epidermolysis bullosa

Q81.9 Epidermolysis bullosa, unspecified

Q82.8 Other specified congenital malformations of skin

Q82.9 Congenital malformation of skin, unspecified

R53.82 Chronic fatigue, unspecified

T86.00 Unspecified complication of bone marrow transplant

T86.01 Bone marrow transplant rejection

T86.03 Bone marrow transplant infection

T86.09 Other complications of bone marrow transplant

T86.10 Unspecified complication of kidney transplant

T86.11 Kidney transplant rejection

T86.13 Kidney transplant infection

T86.19 Other complication of kidney transplant

T86.20 Unspecified complication of heart transplant

T86.21 Heart transplant rejection

T86.23 Heart transplant infection

T86.290 Cardiac allograft vasculopathy

T86.298 Other complications of heart transplant

T86.30 Unspecified complication of heart-lung transplant

T86.31 Heart-lung transplant rejection

T86.33 Heart-lung transplant infection

T86.39 Other complications of heart-lung transplant

T86.40 Unspecified complication of liver transplant

T86.41 Liver transplant rejection

T86.43 Liver transplant infection

T86.49 Other complications of liver transplant

T86.5 Complications of stem cell transplant

T86.810 Lung transplant rejection

T86.812 Lung transplant infection

30

T86.818 Other complications of lung transplant

T86.819 Unspecified complication of lung transplant

T86.830 Bone graft rejection

T86.831 Bone graft failure

T86.832 Bone graft infection

T86.838 Other complications of bone graft

T86.839 Unspecified complication of bone graft

T86.850 Intestine transplant rejection

T86.852 Intestine transplant infection

T86.858 Other complications of intestine transplant

T86.859 Unspecified complication of intestine transplant

T86.890 Other transplanted tissue rejection

T86.892 Other transplanted tissue infection

T86.898 Other complications of other transplanted tissue

T86.899 Unspecified complication of other transplanted tissue

T86.90 Unspecified complication of unspecified transplanted organ and tissue

T86.91 Unspecified transplanted organ and tissue rejection

T86.93 Unspecified transplanted organ and tissue infection

T86.99 Other complications of unspecified transplanted organ and tissue

Z21 Asymptomatic human immunodeficiency virus [HIV] infection status

Z48.21 Encounter for aftercare following heart transplant

Z48.22 Encounter for aftercare following kidney transplant

Z48.23 Encounter for aftercare following liver transplant

Z48.24 Encounter for aftercare following lung transplant

Z48.280 Encounter for aftercare following heart-lung transplant

Z48.288 Encounter for aftercare following multiple organ transplant

Z48.298 Encounter for aftercare following other organ transplant

Z94.0 Kidney transplant status

Z94.1 Heart transplant status

Z94.2 Lung transplant status

Z94.3 Heart and lungs transplant status

Z94.4 Liver transplant status

Z94.82 Intestine transplant status

Z94.83 Pancreas transplant status

Z94.89 Other transplanted organ and tissue status

Z94.9 Transplanted organ and tissue status, unspecified

Facility codingICD-9-CMprocedurecodes: Code Description99.14 Injection or infusion of immunoglobulin

Other Information

Preferred Home Infusion Therapy NetworkReferring providers are encouraged to use these preferred Home Infusion providers to obtain thesemedications.

Preferred Home Infusion Therapy Provider Contact Information:

31

Accredo Health Group

Phone: 1-877-988-0058

Website: www.accredo.com

Caremark, LLC.

Phone: 1-866-846-3096

Website: www.caremark.com

Coram™ Specialty Infusion Services

Phone: 1-800-678-3442

Website: www.coramhc.comHome Solutions

Falmouth Location:Phone: 1-508-548-4266 or toll free 1-800-244-1227

Canton Location:Phone: 1-617-989-0888 or toll free at 1-888-660-1660

Website: www.infusionreferral.com

Individual ConsiderationAll our medical policies are written for the majority of people with a given condition. Each policy is basedon medical science. For many of our medical policies, each individual’s unique clinical circumstancesmay be considered in light of current scientific literature. Physicians may send relevant clinical informationfor individual patients for consideration to:

Blue Cross Blue Shield of MassachusettsClinical Pharmacy DepartmentOne Enterprise DriveQuincy, MA 02171Tel: 1-800-366-7778Fax: 1-800-583-6289

Managed Care Authorization Instructions Prior authorization is required for all out patient sites of service For all outpatient sites of service, physicians may fax or mail the attached form to the address above For all outpatient sites of service, physicians may also submit authorization requests via the web

using Express PAth which can be found on the BCBSMA provider portal or directly on the web athttps://provider.express-path.com

PPO and Indemnity Authorization Instructions Prior authorization is required when this medication is processed under the home infusion therapy

benefit. Prior authorization is not required when this medication is purchased by the physician and

administered in the office in accordance with this medical policy. Physicians may also fax or mail the attached form to the address above. Physicians may also submit authorization requests via the web using Express PAth which can be

found on the BCBSMA provider portal or directly on the web at https://provider.express-path.com

Policy HistoryDate Action7/2014 Updated Coding section with ICD10 procedure and diagnosis codes, effective 10/2015.

32

1/2014 Updated ExpressPAth Language.1/2013 Updated 1/2013 to include new FDA products Gammaked™ and Gamunex®-C.11/2011-4/2012

Medical policy ICD 10 remediation: Formatting, editing and coding updates.No changes to policy statements.

1/2012 Reviewed - Medical Policy Group - Neurology and Neurosurgery.No changes to policy statements.

11/2011 Reviewed - Medical Policy Group - Plastic Surgery and Dermatology.No changes to policy statements.

10/2011 Reviewed - Medical Policy Group - Gastroenterology, Nutrition and OrganTransplantation.No changes to policy statements.

9/2011 Reviewed - Medical Policy Group - Urology and Obstetrics/Gynecology.No changes to policy statements.

1/2011 Reviewed - Medical Policy Group - Neurology and Neurosurgery.No changes to policy statements.

12/2010 Reviewed - Medical Policy Group - Plastic Surgery and Dermatology.No changes to policy statements.

11/2010 Reviewed - Medical Policy Group - Gastroenterology, Nutrition and OrganTransplantation.No changes to policy statements.

10/2010 Reviewed - Medical Policy Group - Urology and Obstetrics/Gynecology.No changes to policy statements.

9/2010 Reviewed - Medical Policy Group - Hematology and Oncology.No changes to policy statements.

1/2010 Reviewed - Medical Policy Group - Neurology and Neurosurgery.No changes to policy statements.

12/2009 Reviewed - Medical Policy Group - Plastic Surgery and Dermatology.No changes to policy statements.

11/2009 Reviewed - Medical Policy Group - Gastroenterology, Nutrition and OrganTransplantation.No changes to policy statements.

10/2009 Reviewed - Medical Policy Group - Urology and Obstetrics/Gynecology.No changes to policy statements.

9/2009 Reviewed - Medical Policy Group - Hematology and Oncology.No changes to policy statements.

10/2009 Updated to reflect UM requirements.1/2009 Reviewed - Medical Policy Group - Neurology and Neurosurgery.

No changes to policy statements.12/2008 Reviewed - Medical Policy Group - Plastic Surgery and Dermatology.

No changes to policy statements.11/2008 Reviewed - Medical Policy Group - Gastroenterology, Nutrition and Organ

Transplantation.No changes to policy statements.

10/2008 Reviewed - Medical Policy Group - Urology and Obstetrics/Gynecology.No changes to policy statements.

10/2008 Reviewed - Medical Policy Group - Hematology and Oncology.No changes to policy statements.

1/2008 Reviewed - Medical Policy Group - Neurology and Neurosurgery.No changes to policy statements.

9/2007 Reviewed - Medical Policy Group - Hematology and Oncology.No changes to policy statements.

1/2007 Reviewed - Medical Policy Group - Neurology and Neurosurgery.No changes to policy statements.

33

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plasma exchange in Guillain-Barre syndrome. N Engl J Med 1992; 326(17):1123-9.2. Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group. Randomised trial of plasma

exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barre syndrome. Lancet1997; 349(9047):225-30.

3. Hahn AF, Bolton CF, Zochodne D et al. Intravenous immunoglobulin treatment in chronicinflammatory demyelinating polyneuropathy. A double-blind, placebo-controlled, cross-over study.Brain 1996; 119(pt 4):1067-77.

4. Sharma KR, Cross J, Ayyar DR et al. Diabetic demyelinating polyneuropathy responsive tointravenous immunoglobulin therapy. Arch Neurol 2002; 59(5):751-7.

5. Dyck PJ, Litchy WJ, Kratz KM et al. A plasma exchange versus immune globulin infusion trial inchronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 1994; 36(6):838-45.

6. Dalakas MC, Quarles RH, Farrer RX et al. A controlled study of intravenous immunoglobulin indemyelinating neuropathy with IgM gammopathy. Ann Neurol 1996; 40(5):792-5.

7. Comi G, Roveri L, Swan A et al. A randomised controlled trial of intravenous immunoglobulin in IgMparaprotein associated with demyelinating neuropathy. J Neurol 2002; 249(10):1370-7.

8. Azulay JP, Blin O, Pouget J et al. Intravenous immunoglobulin treatment in patients with motorneuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study.Neurology 1994; 44(3 pt 1):429-32.

9. Leger JM, Chassande B, Musset L et al. Intravenous immunoglobulin therapy in multifocal motorneuropathy: a double-blind, placebo-controlled study. Brain 2001; 124(pt 1):145-53.

10. Federico P, Zochodne DW, Hahn AF et al. Multifocal motor neuropathy improved by IVIg:randomized, double-blind, placebo-controlled study. Neurology 2000; 55(9):1256-62.

11. Gajdos P, Chevret S, Clair B et al. Clinical trial of plasma exchange and high-dose intravenousimmunoglobulin in myasthenia gravis. Myasthenia Gravis Clinical Study Group. Ann Neurol 1997;41(6):789-96.

12. Qureshi AI, Choudhry MA, Akbar MS et al. Plasma exchange versus intravenous immunoglobulintreatment in myasthenic crisis. Neurology 1999; 52(3):629-32.

13. Ronager J, Ravnborg M, Hermansen I et al. Immunoglobulin treatment versus plasma exchange inpatients with chronic moderate to severe myasthenia gravis. Artif Organs 2001; 25(12):967-73.

14. Selcen D, Dabrowski ER, Michon AM et al. High-dose intravenous immunoglobulin therapy in juvenilemyasthenia gravis. Pediatr Neurol 2000; 22(1):40-3.

15. 1998 TEC Assessments; Tab 19.16. Goodin DS, Frohman EM, Garmany GP et al. Disease modifying therapies in multiple sclerosis.

Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy ofNeurology and the MS Council for Clinical Practice Guidelines. Neurology 2002; 58(2):169-78.

17. Dalakas MC, Illa I, Dambrosia JM et al. A controlled trial of high-dose intravenous immune globulininfusions as treatment for dermatomyositis. N Engl J Med 1993; 329(27):1993-2000.

18. Al-Mayouf SM, Laxer RM, Schneider R et al. Intravenous immunoglobulin therapy for juveniledermatomyositis: efficacy and safety. J Rheumatol 2000; 27(10):2498-503.

19. Gottfried I, Seeber A, Anegg B et al. High dose intravenous immunoglobulin (IVIG) indermatomyositis: clinical responses and effect on sIL-2R levels. Eur J Dermatol 2000; 10(1):29-35.

20. Cherin P, Pelletier S, Teixeira A et al. Results and long-term follow-up of intravenous immunoglobulininfusions in chronic, refractory polymyositis: an open study with thirty-five adult patients. ArthritisRheum 2002; 46(2):467-74.

21. Medicare coverage policy #CAG-00109N, 2002. Available online at: http://cms.hhs.gov/coverage/8b3-kkk.asp.

22. Bachot N, Revuz J Roujeau JC. Intravenous immunoglobulin treatment for Stevens-Johnsonsyndrome and toxic epidermal necolysis: a prospective noncomparative study showing no benefit onmortality or progression. Arch Dermatol 2003; 139(1):33-6.

23. Letko E, Miserocchi E, Daoud YJ et al. A nonrandomized comparison of the clinical outcome of ocularinvolvement in patients with mucous membrane (cicatricial) pemphigoid between conventionalimmunosuppressive and intravenous immunoglobulin therapies. Clin Immunol 2004; 111(3):303-10.

34

24. Dalakas MC, Sonies B, Dambrosia J et al. Treatment of inclusion-body myositis with IVIg: a double-blind, placebo-controlled study. Neurology 1997; 48(3):712-6.

25. Walter MC, Lochmuller H, Toepfer M et al. High-dose immunoglobulin therapy in sporadic inclusionbody myositis: a double-blind, placebo-controlled study. J Neurol 2000; 247(1):22-8.

26. Dalakas MC, Koffman B, Fujii M et al. A controlled study of intravenous immunoglobulin combinedwith prednisone in the treatment of IBM. Neurology 2001; 56(3):323-7.

27. Newburger JW, Takahashi M, Beiser AS et al. A single intravenous infusion of gamma globulincompared with four infusions in the treatment of acute Kawasaki syndrome. N Engl J Med 1991;324(23):1633-9.

28. Jayne DR, Chapel H, Adu D et al. Intravenous immunoglobulin for ANCA-associated systemicvasculitis with persistent disease activity. QJM 2000; 93(7):433-9.

29. Lockwood CM. New treatment strategies for systemic vasculitis: the role of intravenous immuneglobulin therapy. Clin Exp Immunol 1996; 104(suppl 1):77-82.

30. 1998 TEC Assessments; Tab 14.31. Bussel JB, Berkowitz RL, Lynch L et al. Antenatal management of alloimmune thrombocytopenia with

intravenous immunoglobulin: a randomized trial of the addition of low-dose steroid to intravenousgamma-globulin. Am J Obstet Gynecol 1996; 174(5):1414-23.

32. Kiehl MG, Stoll R, Broder M et al. A controlled trial of intravenous immune globulin for the preventionof serious infections in adults with advanced human immunodeficiency virus infection. Arch InternMed 1996; 156(22):2545-50.

33. Vollmer-Conna U, Hickie I, Hadzi-Pavlovic D et al. Intravenous immunoglobulin is ineffective in thetreatment of patients with chronic fatigue syndrome. Am J Med 1997; 103(1):38-43.

34. Kress HG, Scheidewig C, Schmidt H et al. Reduced incidence of postoperative infection afterintravenous administration of an immunoglobulin A- and immunoglobulin M-enriched preparation inanergic patients undergoing cardiac surgery. Crit Care Med 1999; 27(7):1281-7.

35. Douzinas EE, Pitaridis MT, Louris G et al. Prevention of infection in multiple trauma patients by high-dose intravenous immunoglobulins. Crit Care Med 2000; 28(1):8-15.

36. Voss LM, Wilson NJ, Neutze JM et al. Intravenous immunoglobulin in acute rheumatic fever: arandomized controlled trial. Circulation 2001; 103(3):401-6.

37. McNamara DM, Holubkov R, Starling RC et al. Controlled trial of intravenous immune globulin inrecent-onset dilated cardiomyopathy. Circulation 2001; 103(18):2254-9.

38. Hundt M, Manger K, Dorner T et al. Treatment of acute exacerbation of systemic lupuserythematosus with high-dose intravenous immunoglobulin. Rheumatology (Oxford) 2000;39(11):1301-2.

39. Levy Y, Sherer Y, Ahmed A et al. A study of 20 SLE patients with intravenous immunoglobulin--clinical and serologic response. Lupus 1999; 8(9):705-12.

40. Boletis JN, Ioannidis JP, Boki KA et al. Intravenous immunoglobulin compared withcyclophosphamide for proliferative lupus nephritis. Lancet 1999; 354(9178):569-70.

41. Casadei DH, del C Rial M, Opelz G et al. A randomized and prospective study comparing treatmentwith high-dose intravenous immunoglobulin with monoclonal antibodies for rescue of kidney graftswith steroid-resistant rejection. Transplantation 2001; 71(1):53-8.

42. Luke PP, Scantlebury VP, Jordan ML et al. Reversal of steroid- and anti-lymphocyte antibody-resistant rejection using intravenous immunoglobulin (IVIG) in renal transplant recipients.Transplantation 2001; 72(3):419-22.

43. Jordan SC, Quartel AW, Czer LS et al. Posttransplant therapy using high-dose humanimmunoglobulin (intravenous gammaglobulin) to control acute humoral rejection in renal and cardiacallograft recipients and potential mechanism of action. Transplantation 1998; 66(6):800-5.

44. LeHoang P, Cassoux N, George F et al. Intravenous immunoglobulin (IVIg) for the treatment ofbirdshot retinochoroidopathy. Ocul Immunol Inflamm 2000; 8(1):49-57.

45. Rosenbaum JT, George RK, Gordon C. The treatment of refractory uveitis with intravenousimmunoglobulin. Am J Ophthalmol 1999; 127(5):545-9.

46. Noseworthy JH, O'Brien PC, Petterson TM et al. A randomized trial of intravenous immunoglobulin ininflammatory demyelinating optic neuritis. Neurology 2001; 56(11):1514-22.

47. Kishiyama JL, Valacer D, Cunningham-Rundles C et al. A multicenter, randomized, double-blind,placebo-controlled trial of high-dose intravenous immunoglobulin for oral corticosteroid-dependentasthma. Clin Immunol 1999; 91(2):126-33.

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48. Salmun LM, Barlan I, Wolf HM et al. Effect of intravenous immunoglobulin on steroid consumption inpatients with severe asthma: a double-blind, placebo-controlled, randomized trial. J Allergy ClinImmunol 1999; 103(5 pt 1):810-5.

49. Sullivan KM, Kopecky KJ, Jocom J et al. Immunoglobulin and antimicrobial efficacy of intravenousimmunoglobulin in bone marrow transplantation. N Engl J Med 1990; 323(11):705-12.

50. Bass EB, Powe NR, Goodman SN et al. Efficacy of immune globulin in preventing complications ofbone marrow transplantation: a meta-analysis. Bone Marrow Transplant 1993; 12(3):273-82.

51. Guglielmo BJ, Wong-Beringer A, Linker CA. Immune globulin therapy in allogeneic bone marrowtransplant: a critical review. Bone Marrow Transplant 1994; 13(5):499-510.

52. American Society of Reproductive Medicine. Intravenous Immunoglobulin (IVIG) and RecurrentSpontaneous Pregnancy Loss: A Practice Committee Report; A Committee Opinion. 1998. Availableat http://www.asrm.org/Media/Practice/ivig.html. Accessed October 2002.

53. Scott JR. Immunotherapy for recurrent miscarriage (Cochrane Review). In: The Cochrane Library,Issue 3, 2002. Oxford: Update Software.

54. Jablonowska B, Selbing A, Palfi M et al. Prevention of recurrent spontaneous abortion by intravenousimmunoglobulin: a double-blind placebo-controlled study. Hum Reprod 1999; 14(3):838-41.

55. Branch DW, Peaceman AM, Druzin M et al. A multicenter, placebo-controlled pilot study ofintravenous immune globulin treatment of antiphospholipid syndrome during pregnancy. ThePregnancy Loss Study Group. Am J Obstet Gynecol 2000; 182(1 Pt 1):122-7.

56. Christiansen OB, Pedersen B, Rosgaard A et al. A randomized, double-blind, placebo-controlled trialof intravenous immunoglobulin in the prevention of recurrent miscarriage: evidence for a therapeuticeffect in women with secondary recurrent miscarriage. Hum Reprod 2002; 17(3):809-16.

57. Cordonnier C, Chevret S, Legrand M et al. Should immunoglobulin therapy be used in allogeneicstem-cell transplantation? A randomized, double-blind, dose effect, placebo-controlled multicentertrial. Ann Intern Med 2003; 139(1):8-18.

58. Jordan SC, Tyan D, Stablein D et al. Evaluation of intravenous immunoglobulin as an agent to lowerallosensitization and improve transplantation in highly sensitized adult patients with end-stage renaldisease: report of the NIH IG02 trial. J Am Soc Nephrol 2004; 15(12):3256-62.

59. Jordan SC, Vo AA, Nast CC et al. Use of high-dose human intravenous immunoglobulin therapy insensitized patients awaiting transplantation: the Cedars-Sinai experience. Clin Transpl 2003; 193-8.

60. MontgomeryRA, Zachary AA. Transplanting patients with a positive donor-specific crossmatch: asingle center’s perspective. Pediatr Transplant 2004; 8(6):535-42.

61. Jordan SC, Vo AA, Tyan D et al. Current approaches to treatment of antibody-mediated rejection.Pediatr Transplant 2005; 9(3):408-15.

62. Lehrich RW, Rocha PN, Reinsmoen N et al. Intravenous immunoglobulin and plasmapheresis inacute humoral rejection: Experience in renal allograft transplantation. Hum Immunol 2005; 66(4):350-8.

63. Casadei DH, del C Rial M, Opelz G et al. A randomized and prospective study comparing treatmentwith high-dose intravenous immunoglobulin with monoclonal antibodies for rescue of kidney graftswith steroid-resistant rejection. Transplantation 2001; 71(1):53-8.

64. Ibernon M, Gil-Vernet S, Carrera M et al. Therapy with plasmapheresis and intravenousimmunoglobulin for acute humoral rejection in kidney transplantation. Transplant Proc 2005;37(9):3743-5.

Endnotes1. Revised 9/95 based on TEC (Technology Evaluation Center) 6/95 assessment of medical literature

from 1991 to 1995 addressing IVIg for SLE-related cytopenia, vasculitis, pericarditis, and pleuraleffusions in patients who were not controlled by immunosuppressives or cytotoxic agents.

2. Revised 9/95 to include the 2/95 TEC evaluation of medical literature from 1991-4/95 assessing IVIgto improve the functional status of patients with inclusion body myositis who have not responded toprednisone or other immunosuppressives.

3. Revised 10/95 based on 1994 TEC evaluation of medical literature from 1991-1994 assessing IVIG tostop progression of muscle weakness or to decrease frequency or severity of relapses in MS..

36

4. Revised 10/95 based on a 1994 TEC evaluation of medical literature from 1991-1994 assessing IVIgto improve functional capacity or to reduce pain in patients with RA refractory to NSAIDS and eithercytotoxic or disease-modifying antirheumatic drugs.

5. Revised 10/95 based on a 1994 TEC evaluation of medical literature from 1991-1994 assessing IVIGto improve neurologic function in CIDP, either as first-line therapy, or for acute exacerbations inpatients refractory or intolerant of prednisone or azathioprine.

6. Revised 10/95 based on a 1994 TEC evaluation of medical literature assessing IVIG to reduce fetalloss in women with recurrent fetal loss (sequence of 3 or more miscarriages), with or withoutantiphospholipid antibodies.

7. Revised 3/96 to include CMS (Centers for Medicare and Medicaid services) regulations published inthe February/March 1996 issue of the Medicare Health Resources.

8. Revised 2/97 to include CMS (Centers for Medicare and Medicaid services) regulations published inthe February/March 1997 issue of the Medicare Health Resources.

9. Revised 9/97 to include CMS regulations (Centers for Medicare and Medicaid services) published inthe June/July 1997 Medicare B Health Resources.

10. Added based on recommendations made by the Massachusetts Neurological Society.11. Based on the July 1998 TEC (Technology Evaluation Center) analysis of the literature on IVIg for

MS. Health outcomes considered by TEC included prevention of disease progress and disability,improving baseline neuro disability, and reducing acute relapse.Also see the July/August 1997 ACP Journal Club commentary:http://www.acponline.org/journals/acpjc/julaug97Regarding the article: Fazekas F et al., Austrian Immunoglobulin in Multiple Sclerosis Study Group.Randomized placebo-controlled trial of monthly intravenous immunoglobulin therapy in relapsing-remitting multiple sclerosis. Lancet. 1997 Mar 1;349:589-93.

12. FDA-approved uses as of July, 1998.13. Off-label use in the treatment of AIDS and HIV as required by law.14. Label use based on National Blue Cross Blue Shield policy 8.01.05, issued 12/15/98.15. Off-label use based on National Blue Cross Blue Shield policy 8.01.05, issued 12/15/98.16. Investigational use based on National Blue Cross Blue Shield policy 8.01.05, issued 12/15/98.17. Based on recommendations from Walt Kagan, MD, Massachusetts Society of Clinical Oncologists.18. Based upon a September 1999 Medicare B HealthResource Newsletter.19. Medicare policy is developed separately from BCBSMA policy. While BCBSMA policy is based upon

scientific evidence, Medicare policy incorporates scientific evidence with local expert opinion, andgovernmental regulations from CMS (Centers for Medicare and Medicaid Services) and the U.SCongress. While BCBSMA and Medicare policies may differ, our Medicare HMO Blue and MedicarePPO Blue members must be offered the same services as Medicare offers. In many instances,BCBSMA policies offer more benefits than does Medicare policy.

20. Based on recommendations from David Weinberg, MD, Massachusetts Neurologic Association,1/2000 MPG Neurology meeting.

21. Medical Policy Group, August 2000.22. Previous criteria summarized in the current form: vital capacity less than 1L; dysphagia associated

with aspiration; inability to ambulate 100 feet without assistance.23. Medical Policy Group, January 2000.24. Idiopathic Thrombocytopenic Purpura: A Practice Guideline Developed by Explicit Methods for the

American Society of Hematology25. See the 1998 ASRM (American Society of Reproductive Medicine) Practice Committee Report on

Intravenous Immunoglobulin and Spontaneous Pregnancy Loss.26. Based on the June 2002 Medicare B Resource Newsletter. See also the CMS /Medicare websites at

www.cms.gov and www medicare.gov.27. Based upon the 2002 Blue Cross Blue Shield Association policy 8.01.05. IVIG for myasthenic crisis

is considered medically necessary. Myasthenic crisis is an off-label indication.28. Based upon the 2002 Blue Cross Blue Shield Association National policy 8.01.05.29. Based upon the 2004 Blue Cross Blue Shield Association policy 2.01.01.30. Based upon the 2004 Blue Cross Blue Shield Association National policy 8.01.05.31. Consensus statement on the use of intravenous immunoglobulin therapy in the treatment of

autoimmune mucocutaneous blistering diseases. Arch Dermatol.2003;139:1051-1059.

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32. Based upon the 2004 BCBSA National Policy 8.01.05. Bone marrow transplant patients (forprevention of infection or GVH prevention.) Cordonnier C, Chevret S, Legrand M et al. Should immunoglobulin therapy be used in allogeneic

stem-cell transplantation? A randomized, double-blind, dose effect, placebo-controlled multicentertrial. Ann Intern Med 2003;139(1):8-18.

33. Based upon the 2004 BCBSA National Policy 8.01.05. Recurrent Spontanous Abortion.34. Based on Blue Cross Blue Shield National policy 8.01.05 Intravenous Immune Globulin Therapy

issued 4/06.

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Home Infusion TherapyPrior Authorization Form

Please complete and fax with the physician's prescription to: (888) 641-5355. If the patient is aBCBSMA employee, please fax the form to: (617)246-4013.

Companyname:

ContactName:

Phone #: Provider #:

Fax# Address:

Patientname:

Address:

Patient_ID#: DOB:____/____/____ Diagnosis:

PrescribingPhysician/addr:

____________________________________ Telephone:

PCP name/address: ____________________________________ Telephone:

Is this fax number ‘secure’ for PHI receipt/transmission per HIPAA requirements? (circle one) Yes No

Place of Service Home SNF MD office other (specify)_____________________Primary Therapy

Primary drugname:

Approximateduration:

____/____/____ to____/____/____

Dose:

Frequency: Route of Administration: pump: Y N

Other Therapy

Other drug name: Approximateduration:

____/____/____ to____/____/____

Dose:

Frequency Route of Administration: pump: Y N

If this is a “drug only” authorization request, indicate other services the nursing agency is providing:

______________________________________________________________________________________Nursing provided by: ________________________________ Contact: _________________________Phone: ______________ Fax: ___________________Request for 7 Day Coverage : Date of occurrence: ___________ request dates:___________________

Occurrence type: Hospitalization Death Change of Therapy

Physician signature:_______________________________________________ Date:____________________

OR Copy of prescription REQUIRED with this request

39

Outpatient Medical Prior Authorization FormPlease complete and fax to: (888) 641-5355

Please contact Pharmacy Operations with questions at (800) 366-7778If the patient is a BCBSMA employee, please fax the form to: (617) 246-4013

Servicing

Provider

Name:

NPI Number:

Requesting

Provider

Name:

NPI Number:

Phone#

Fax#

Contact

Person:

Phone#

Fax#

Contact

Person:

Patient Name: DOB___/___/___ Diagnosis:

Patient

BCBSMA ID#

Is this fax number ‘secure’ for PHI receipt/transmission per HIPAA requirements? (circle one) Yes No

Drug/Therapy:Drug Name:_____________________________________ Dates of Service:___/___/___ to ___/___/___

Dose:________________________________________________________________________________

Frequency:____________________________________________________________________________

Additional Clinical (including previous treatment failure):

Physician signature:___________________________________________ Date:_____________________