Dental Dental Management of Management of

Patients withPatients withRheumatology Rheumatology

DisordersDisorders22

Systemic Lupus Systemic Lupus ErythematosusErythematosus

A chronic multisystem disease of A chronic multisystem disease of unknown origin that exhibits wide unknown origin that exhibits wide

variations in its clinical expression and variations in its clinical expression and disease coursedisease course

Females are affected 8-10 times more than Females are affected 8-10 times more than menmen

Average age is 31 yearsAverage age is 31 years

Signs and SymptomsSigns and Symptoms

Fever, weight loss, Fever, weight loss, arthritis, fatigue, arthritis, fatigue, and general and general malaisemalaise

Butterfly rash on Butterfly rash on the malar area and the malar area and nosenose

40-50% of patients 40-50% of patients have affected have affected kidneyskidneys

Cardiac Cardiac involvementinvolvement-Libman-sacks endocarditis -Libman-sacks endocarditis found in 50% of patients upon found in 50% of patients upon autopsyautopsy

-pericarditis, myocarditis, -pericarditis, myocarditis, endocarditis, CADendocarditis, CAD

Oral lesionsOral lesions-5-25% of patients have -5-25% of patients have affected palate, buccal affected palate, buccal mucosa, and gingivamucosa, and gingiva

SLE : Classification CriteriaSLE : Classification Criteria

1. 1. SSerositis: erositis: (a) pleuritis, or (a) pleuritis, or (b) pericarditis(b) pericarditis

2. 2. OOral ulcersral ulcers3. 3. AArthritisrthritis4. 4. PPhotosensitivityhotosensitivity

10. 10. MMalar rashalar rash11. 11. DDiscoid rashiscoid rash

5.5. B Blood/Hematologic disorder: lood/Hematologic disorder: (a) hemolytic anemia or(a) hemolytic anemia or(b) leukopenia of < 4.0 x 10(b) leukopenia of < 4.0 x 1099 (c) lymphopenia of < 1.5 x (c) lymphopenia of < 1.5 x

101099 (d) thrombocytopenia < 100 (d) thrombocytopenia < 100

X 10X 1099

6. 6. RRenal disorder: enal disorder: (a) proteinuria > 0.5 gm/24 h (a) proteinuria > 0.5 gm/24 h

or or 3+ dipstick or3+ dipstick or(b) cellular casts(b) cellular casts

7. 7. AAntinuclear antibody (positive ANA) ntinuclear antibody (positive ANA) 8. 8. IImmunologic disorders: mmunologic disorders:

(a) raised anti-native DNA (a) raised anti-native DNA antibody binding orantibody binding or(b) anti-Sm antibody or (b) anti-Sm antibody or (c) positive anti-phospholipid (c) positive anti-phospholipid antibody work-upantibody work-up

9. 9. NNeurological disorder: eurological disorder: (a) seizures or (a) seizures or (b) psychosis(b) psychosis

". ..A person shall be said to have SLE if four or more of the 11 criteria are present, serially or simultaneously, during any interval of observation."

Systemic Lupus ErythematosusSystemic Lupus Erythematosus Head and Neck ManifestationsHead and Neck Manifestations

Malar rash first sign in 50%Malar rash first sign in 50% Erythematous maculopapular eruption after sun Erythematous maculopapular eruption after sun

exposureexposure Oral ulcerationOral ulceration 3-5% nasal septum perforation3-5% nasal septum perforation Acute parotid enlargement 10%Acute parotid enlargement 10% Xerostomia 15%Xerostomia 15% Larynx and trachea involvement uncommonLarynx and trachea involvement uncommon

-Vocal Cords thickening and paralysis, -Vocal Cords thickening and paralysis, cricoarytenoid arthritis, subglottic stenosiscricoarytenoid arthritis, subglottic stenosis

Laboratory ValuesLaboratory Values

95% of patients have elevated ANA95% of patients have elevated ANA 70% of patient have antibodies 70% of patient have antibodies

directed against double-stranded directed against double-stranded DNA DNA

Medical & Dental Medical & Dental ManagementManagement

Medical ManagementMedical Management Avoid excessive exposure to sunlightAvoid excessive exposure to sunlight NSAIDS for mild active disease with NSAIDS for mild active disease with

antimalarial drugs antimalarial drugs Topical corticosteroidsTopical corticosteroids Dental ManagementDental Management Assess adrenal function for possible suppressionAssess adrenal function for possible suppression Consult with physician regarding systemic Consult with physician regarding systemic

manifestationmanifestation Assess if NSAIDS are affecting platelet functionAssess if NSAIDS are affecting platelet function

Sjogren SyndromeSjogren Syndrome

Chronic disorder characterized by immune-mediated Chronic disorder characterized by immune-mediated destruction of exocrine glandsdestruction of exocrine glands

A slowly progressive inflammatory disease affecting A slowly progressive inflammatory disease affecting primarily the exocrine glandsprimarily the exocrine glands

1% of the population and in 10-15% of RA patients1% of the population and in 10-15% of RA patients 9:1 Female :Male preponderance9:1 Female :Male preponderance Age of onset 40-60 yearsAge of onset 40-60 years Dry mouth, skin, eyes, nose and vaginaDry mouth, skin, eyes, nose and vagina Tongue becomes fissured and exhibits atrophy of the Tongue becomes fissured and exhibits atrophy of the

papillaepapillae Oral mucosa red and tenderOral mucosa red and tender Parotid enlargementParotid enlargement

Sjogren SyndromeSjogren Syndrome Primary vs Secondary:Primary vs Secondary: Primary is diagnosis of exclusionPrimary is diagnosis of exclusion Secondary refers to the sicca complex Secondary refers to the sicca complex

accompanying any of the connective tissue accompanying any of the connective tissue diseases (xerophthalmia, keratoconjuntivitis, diseases (xerophthalmia, keratoconjuntivitis, xerostomia with/without salivary gland xerostomia with/without salivary gland enlargement)enlargement)

Lymphocytic infiltratesLymphocytic infiltrates Associated with a 33-44 times increased risk Associated with a 33-44 times increased risk

of lymphoma.of lymphoma. Characteristic autoantibodies - Ro, La Characteristic autoantibodies - Ro, La

Extra-glandular Signs and Extra-glandular Signs and SymptomsSymptoms

Raynaud’s phenomenonRaynaud’s phenomenon Peripheral Neuropathies Peripheral Neuropathies LymphadenopathyLymphadenopathy VasculitisVasculitis Interstitial nephritisInterstitial nephritis Interstitial lung fibrosisInterstitial lung fibrosis Primary Biliary CirrhosisPrimary Biliary Cirrhosis

Sjogren SyndromeSjogren Syndrome

Laboratory ValuesLaboratory Values

High ESR & serum immunoglobulin High ESR & serum immunoglobulin levels (IgG)levels (IgG)

75% of patients have RF regardless of 75% of patients have RF regardless of rheumatoid arthritisrheumatoid arthritis

Antinuclear antibodies (ANA) also Antinuclear antibodies (ANA) also presentpresent

Sjogren Syndrome Diagnostic Sjogren Syndrome Diagnostic CriteriaCriteria

1. Dry eyes (>3mos), sensation of sand or gravel in 1. Dry eyes (>3mos), sensation of sand or gravel in eyes, or use of tear substitutes>3x per dayeyes, or use of tear substitutes>3x per day

2. Dry mouth (>3mos), recurrent or persistent 2. Dry mouth (>3mos), recurrent or persistent swollen salivary glands, or frequent drinking of swollen salivary glands, or frequent drinking of liquids to aid in swallowing dry foods.liquids to aid in swallowing dry foods.

3. Schirmer-I test (<5mm in 5 min) or Rose Bengal 3. Schirmer-I test (<5mm in 5 min) or Rose Bengal score >4.score >4.

4. >50 mononuclear cells/4mm4. >50 mononuclear cells/4mm2 2 glandular tissueglandular tissue

5. Abnormal salivary scintigraphy or parotid 5. Abnormal salivary scintigraphy or parotid sialography or unstimulated salivary flow sialography or unstimulated salivary flow <1.5ml in 15 min<1.5ml in 15 min

6. Presence of anti-Ro/SS-A, anti-La/SS-b, 6. Presence of anti-Ro/SS-A, anti-La/SS-b, antinuclear antibodies, or rheumatoid factor.antinuclear antibodies, or rheumatoid factor.

Medical ManagementMedical Management Local manifestations can be treated Local manifestations can be treated

symptomaticallysymptomatically Symptomatic: saliva substitutes, Symptomatic: saliva substitutes,

artificial tears, increased oral fluid artificial tears, increased oral fluid intakeintake

Avoid decongestants, antihistamines, Avoid decongestants, antihistamines, anticholinergics, diureticsanticholinergics, diuretics

Pilocarpine, antifungals, close dental Pilocarpine, antifungals, close dental follow-up, surveillance for malignancyfollow-up, surveillance for malignancy

Dental ManagementDental Management

Prevention of cariesPrevention of cariesdaily use of fluoride, frequent recallsdaily use of fluoride, frequent recalls

Enhance salivary outputEnhance salivary outputsugarless gum/candy, saliva substitutes (salivart, biotene, sugarless gum/candy, saliva substitutes (salivart, biotene,

oral oral balance, mouth kote, glandosane, prescription medication balance, mouth kote, glandosane, prescription medication (salagen, `(salagen, ` evoxac)evoxac)

Treatment of oral candidiasisTreatment of oral candidiasisantifungalsantifungals

Pain control for enlarged salivary Pain control for enlarged salivary glandsglands

SclerodermaScleroderma Generalised disorder of CT affecting skin Generalised disorder of CT affecting skin

(scleroderma)(scleroderma) and internal organs and internal organs Associated with specific auto-antibodiesAssociated with specific auto-antibodies Localised (CREST) or Systemic ( Variants Localised (CREST) or Systemic ( Variants

SSc)SSc) May be benign cutaneous involvement or May be benign cutaneous involvement or

aggressive systemic disease.aggressive systemic disease. 4-12 new cases per million per year4-12 new cases per million per year 3-4:1 Female preponderance3-4:1 Female preponderance Average age of onset between 3Average age of onset between 3rdrd and 5 and 5thth

decadedecade

Scleroderma : PresentationScleroderma : Presentation Raynaud’s phenomenonRaynaud’s phenomenon Inflammatory, indurative then atrophic Inflammatory, indurative then atrophic

phasephase Oedema, tightening, thickening, waxy feelOedema, tightening, thickening, waxy feel edema fingers and hands & Loss of function of edema fingers and hands & Loss of function of

handhand skin thickeningskin thickening Arms, legs, chest involvedArms, legs, chest involved

Visceral manifestationsVisceral manifestations GI tract, lung, heart, kidneys, thyroid , arthralgias GI tract, lung, heart, kidneys, thyroid , arthralgias

and muscle weakness oftenand muscle weakness often

Musculoskeletal: Arthralgia, Musculoskeletal: Arthralgia, MyalgiaMyalgia

Small mouth, tightening of skin around mouthVitiligo or hyperpigmentation, telangectasia,

Skin changes in systemic Skin changes in systemic sclerosissclerosis

Raynaud’s Raynaud’s phenomenonphenomenon

Calcinosis Ulceration

Systemic Systemic involvementinvolvement

Gut Gut involvementinvolvement OropharynxOropharynx Esophagus Esophagus Small bowel Small bowel Large bowelLarge bowel

PulmonaryPulmonary

Interstitial Interstitial fibrosisfibrosis

Pulmonary HTNPulmonary HTN

CardiacCardiac

CCF, ArrythmiasCCF, Arrythmias

RenalRenal

Accelerated HTN Accelerated HTN

Renal CrisisRenal Crisis

Head and Neck ManifestationsHead and Neck Manifestations Dysphagia most common initial complaint:Dysphagia most common initial complaint:

80% exhibit pathology in distal 2/3 of esophagus 80% exhibit pathology in distal 2/3 of esophagus Decreased or absent peristalsis, hiatal hernia, Decreased or absent peristalsis, hiatal hernia,

refluxreflux Tight, thin lips with vertical perioral furrowsTight, thin lips with vertical perioral furrows Trismus 2Trismus 2ndnd to tight skin, not TMJ path to tight skin, not TMJ path Xerostomia, xerophthalmia, Xerostomia, xerophthalmia, Laryngeal involvement with hoarsenessLaryngeal involvement with hoarseness Transition zone around dental rootsTransition zone around dental roots

Considered pathognomonic by someConsidered pathognomonic by some

Scleroderma Diagnostic Scleroderma Diagnostic CriteriaCriteria

One major criterion: scleromatous skin One major criterion: scleromatous skin changes proximal to the metacarpal-changes proximal to the metacarpal-phalangeal jointsphalangeal joints

Two of three minor criteria: Two of three minor criteria: sclerodactyly, digital pitting scars, bi-sclerodactyly, digital pitting scars, bi-basilar pulmonary fibrosis on CXRbasilar pulmonary fibrosis on CXR

InvestigationInvestigation BP - monitor closely for hypertensionBP - monitor closely for hypertension Renal function - Creatinine clearance Renal function - Creatinine clearance ANA, anti- ENA, anti-centromereANA, anti- ENA, anti-centromere

Pulmonary function testsPulmonary function tests ECG ECG Radiology Radiology

CXR, CT scan chest, barium swallowCXR, CT scan chest, barium swallow

ManagementManagement Raynaud’sRaynaud’s

Simple measuresSimple measures vasodilators - topical (nitrate), oral vasodilators - topical (nitrate), oral

(calcium channel blockers) (calcium channel blockers) Skin diseaseSkin disease

Simple measures, Immunosuppressive Simple measures, Immunosuppressive Systemic involvementSystemic involvement

Specific therapy aimed at organ involvedSpecific therapy aimed at organ involved Prevent renal crisis with ACE InhibitionPrevent renal crisis with ACE Inhibition

Prognosis: systemic Prognosis: systemic sclerosissclerosis

5 yr survival5 yr survival 70-90%70-90% 10 yr survival10 yr survival 50-70%50-70%

Causes of deathCauses of death:: Renal CrisisRenal Crisis

PulmonaryPulmonary HHypertensionypertension

Myocardial IschaemiaMyocardial Ischaemia

Polymyositis and Polymyositis and DermatomyositisDermatomyositis

Chronic inflammation in striated muscleChronic inflammation in striated muscle Proximal muscle weakness Proximal muscle weakness 2 main subgroups recognised2 main subgroups recognised

Myositis Myositis Dermatomyositis (with skin involvement)Dermatomyositis (with skin involvement)

5 cases per million per year5 cases per million per year 2:1 Female: Male2:1 Female: Male Age 40-60, but a pediatric variant of 5-15 year Age 40-60, but a pediatric variant of 5-15 year

oldold Autoantibody associationAutoantibody association

anti-Jo-1, anti-Mi-2anti-Jo-1, anti-Mi-2

Head and Neck ManifestationsHead and Neck Manifestations Difficulty phonating and deglutition 2Difficulty phonating and deglutition 2ndnd to to

affected tongue musculatureaffected tongue musculature Nasal regurgitation 2Nasal regurgitation 2ndnd to affected to affected

pharyngeal and palatal musculaturepharyngeal and palatal musculature 30% with dysphagia 230% with dysphagia 2ndnd to involvement of to involvement of

upper esophagus, cricopharyngeus, pharynx upper esophagus, cricopharyngeus, pharynx Aspiration pneumoniaAspiration pneumonia

Skin RashesSkin RashesHeliotrope rashHeliotrope rash around around

eyeseyes

Gottron’s papules on Gottron’s papules on knucklesknuckles

Machinists handsMachinists hands

Investigations - MyositisInvestigations - Myositis

Blood testsBlood tests CPK, AST, ALT, LDH, ESRCPK, AST, ALT, LDH, ESR ANA (80%), anti-synthetase antibodies - ANA (80%), anti-synthetase antibodies -

anti-Jo-1 (lung), PM-Scl (SSc), RNP (SLE) anti-Jo-1 (lung), PM-Scl (SSc), RNP (SLE) ECG ECG Radiology - CXR, MRI of thigh muscleRadiology - CXR, MRI of thigh muscle EMGEMG Biopsy Biopsy - muscle, skin- muscle, skin Investigation for malignancy in older age Investigation for malignancy in older age

groupgroup

DiagnosisDiagnosis Proximal muscle weaknessProximal muscle weakness Elevated serum creatinine kinaseElevated serum creatinine kinase Myopathic changes on Myopathic changes on

electromyographyelectromyography Muscle biopsy with evidence of Muscle biopsy with evidence of

lymphocytic inflammationlymphocytic inflammationDiagnosis Diagnosis is :is : Definitive with all fourDefinitive with all four Probable with threeProbable with three Possible with two.Possible with two.

Rash accompanies these in Rash accompanies these in dermatomyositisdermatomyositis

Therapy - MyositisTherapy - Myositis

CorticosteroidsCorticosteroids oral, IV pulseoral, IV pulse

ImmunosuppressantsImmunosuppressants methotrexate, azathioprine, cyclosporin methotrexate, azathioprine, cyclosporin

AA Immunoglobulin (IV IG)Immunoglobulin (IV IG) Monitor disease progressMonitor disease progress

CPK, muscle strengthCPK, muscle strength

Giant Cell Arteritis (Temporal Giant Cell Arteritis (Temporal Arteritis)Arteritis)

Most common vasculitisMost common vasculitis Prevalence:850/100000Prevalence:850/100000 Age 80+Age 80+ Only extracranial vessels involvedOnly extracranial vessels involved Most common initial complaint: Headache-boring and Most common initial complaint: Headache-boring and

constant (47%), up to 90% will develop headacheconstant (47%), up to 90% will develop headache Tender and erythematous temporal artery 50%Tender and erythematous temporal artery 50% Blindness: 1/3 untreated patientsBlindness: 1/3 untreated patients Tender scalpTender scalp Jaw ischemia 50%Jaw ischemia 50% Lingual ischemia 25%Lingual ischemia 25% ESR >50mm/hrESR >50mm/hr Confirmed by temporal artery biopsy of affected sideConfirmed by temporal artery biopsy of affected side Treatment with prednisone and normalizaton of ESRTreatment with prednisone and normalizaton of ESR

Polymyalgia RheumaticaPolymyalgia Rheumatica

Seen in 50% of patients with giant Seen in 50% of patients with giant cell arteritiscell arteritis

Muscular pain, morning stiffness of Muscular pain, morning stiffness of proximal muscles, elevated ESR proximal muscles, elevated ESR without inflammatory joint or muscle without inflammatory joint or muscle diseasedisease

Low grade fever, wt loss, malaiseLow grade fever, wt loss, malaise Low dose prednisoneLow dose prednisone

Relapsing PolychondritisRelapsing Polychondritis GeneralGeneral

Recurring inflammation cartilaginous structuresRecurring inflammation cartilaginous structures Eventual fibrosisEventual fibrosis PrevalencePrevalence

F>MF>M 25-4525-45

Can affect any cartilaginous structureCan affect any cartilaginous structure Including heart valves and large arteriesIncluding heart valves and large arteries

Diagnostic criteriaDiagnostic criteria Recurrent chondritis of the auriclesRecurrent chondritis of the auricles Nonerosive inflammatory polyarthritisNonerosive inflammatory polyarthritis Chondritis of the nasal cartilagesChondritis of the nasal cartilages Inflammation of ocular structuresInflammation of ocular structures Chondritis of laryngeal or tracheal cartilages, Chondritis of laryngeal or tracheal cartilages, Cochlear (SNHL, tinnitus) Vestibular (vertigo) Cochlear (SNHL, tinnitus) Vestibular (vertigo)

damagedamage

PolychondritisPolychondritis Laboratory Laboratory

ESR, leukocytosis, anemiaESR, leukocytosis, anemia HistologyHistology

Loss of basophilic staining of cartilageLoss of basophilic staining of cartilage Perichondral inflammationPerichondral inflammation Destruction fibrotic replacementDestruction fibrotic replacement

TreatmentTreatment Salicylates, ibuprofen-symptomatic reliefSalicylates, ibuprofen-symptomatic relief Steroids for life threateningSteroids for life threatening Dapsone (anti-leprosy) reduces Dapsone (anti-leprosy) reduces

lysozymeslysozymes

Mixed Connective Tissue Mixed Connective Tissue DiseaseDisease

Coexisting features of SLE, Coexisting features of SLE, scleroderma, and polymyositisscleroderma, and polymyositis

High titers of Anti-U1RNPHigh titers of Anti-U1RNP 80% female, 30-60 years80% female, 30-60 years Head and neck: combination of Head and neck: combination of

manifestations of the above. manifestations of the above. Treat with steroidsTreat with steroids

Polyarteritis NodosaPolyarteritis Nodosa Prototype of vasculitisPrototype of vasculitis Less than 1/100000 per yearLess than 1/100000 per year Males = FemalesMales = Females 50-60 years of age50-60 years of age Involves small and medium arteriesInvolves small and medium arteries May result from Hep B infection (30%)May result from Hep B infection (30%) GI, hepatobiliary, renal, pancreas and skeletal GI, hepatobiliary, renal, pancreas and skeletal

musclesmusclesHead and neck symptoms primarily involve the ear Head and neck symptoms primarily involve the ear

and include SNHL and vestibular disturbance.and include SNHL and vestibular disturbance.Proposed mechanism is thromboembolic occlusion Proposed mechanism is thromboembolic occlusion

of inner ear arteriesof inner ear arteriesMay also see CN palsiesMay also see CN palsies

Wegener’s Granulomatosis?Wegener’s Granulomatosis? Young or middle age; M> F Young or middle age; M> F Triad Triad of Necrotizing vasculitis of Upper resp of Necrotizing vasculitis of Upper resp

tract, Lower resp tract, Focal segmental tract, Lower resp tract, Focal segmental glomerulonephritisglomerulonephritis

NonspecificNonspecific

Wegener’s GranulomatosisWegener’s Granulomatosis Upper Respiratory TractUpper Respiratory Tract

Chronic sinusitis with/without hematochezia – 90 %Chronic sinusitis with/without hematochezia – 90 % Oral/nasal ulcers 75 %Oral/nasal ulcers 75 % Otitis media/hearing lossOtitis media/hearing loss Hoarseness of the voiceHoarseness of the voice

Lower Respiratory TractLower Respiratory Tract Cough, Shortness of BreathCough, Shortness of Breath Pulmonary Infiltrates 95 % or Nodules that may Pulmonary Infiltrates 95 % or Nodules that may

cavitatecavitate Renal Renal 80 %80 %

Glomerulonephritis (Rapidly Progressive)Glomerulonephritis (Rapidly Progressive) JointsJoints

ArthritisArthritis

Head and Neck ManifestationsHead and Neck Manifestations Nasal symptomsNasal symptoms

crusting, epistaxis, rhinnorrhea, erosion crusting, epistaxis, rhinnorrhea, erosion of septal cartilage, saddle deformity, of septal cartilage, saddle deformity, recurrent sinusitisrecurrent sinusitis

Oral cavityOral cavity hyperplasia of gingiva, gingivitishyperplasia of gingiva, gingivitis

Wegener’s GranulomatosisWegener’s Granulomatosis Antineutrophil cytoplasmic antibody (c-Antineutrophil cytoplasmic antibody (c-

ANCA)ANCA) sensitivity 65-90%sensitivity 65-90% high specificityhigh specificity

Need to confirm diagnosisNeed to confirm diagnosis often 3-4 biopsies necessaryoften 3-4 biopsies necessary nasopharynx commonly involved good sitenasopharynx commonly involved good site open pulmonary biopsy occasionally open pulmonary biopsy occasionally

neededneeded untreated mortality of 90% at two yearsuntreated mortality of 90% at two years

TreatmentTreatment Meticulous dental and nasal careMeticulous dental and nasal care Middle ear drainageMiddle ear drainage Isolated sinonasal diseaseIsolated sinonasal disease

low dose steroids, saline irrigation, antibiotics as low dose steroids, saline irrigation, antibiotics as neededneeded

Subglottic stenosisSubglottic stenosis may warrant tracheotomymay warrant tracheotomy

Cyclophosphamide 2 mg/kg plus prednisone 1 Cyclophosphamide 2 mg/kg plus prednisone 1 mg/kgmg/kg Remission 93% Remission 93%

Azathioprine or methotrexate alternative to Azathioprine or methotrexate alternative to cyclophosphamidecyclophosphamide

Henoch Schonlein PurpuraHenoch Schonlein Purpura

Purpura on extensor surfaces of arms, legs, buttocks

GI disturbances

Renal involvement

Fever

Previous URI

Hypersensitivity VasculitisHypersensitivity Vasculitis Head and Neck ManifestationsHead and Neck Manifestations

Petechiae, Purpura of oral and nasal Petechiae, Purpura of oral and nasal mucosamucosa

AngioedemaAngioedema Serous otitis mediaSerous otitis media

TreatmentTreatment Usually self limitedUsually self limited

especially when only skin involvedespecially when only skin involved Systemic involvement- more aggressive Systemic involvement- more aggressive

Churg-Strauss SyndromeChurg-Strauss Syndrome

Also called angiitis granulomatosisAlso called angiitis granulomatosis Consists of small vessel vasculitis, Consists of small vessel vasculitis,

extra vascular granulomas, and extra vascular granulomas, and hypereosinophilia.hypereosinophilia.

In patients with preexisting asthma In patients with preexisting asthma and allergic rhinitisand allergic rhinitis

Behcet’s DiseaseBehcet’s Disease

Vasculitis with triad of oral and genital Vasculitis with triad of oral and genital ulcers and uveitis or iritisulcers and uveitis or iritis

Aphthous like ulcers, covered in pale Aphthous like ulcers, covered in pale pseudomembranepseudomembrane

Painful, on lips, gingiva, buccal Painful, on lips, gingiva, buccal mucosa, tongue, palate and mucosa, tongue, palate and oropharynxoropharynx

Genital ulcers similar in appearanceGenital ulcers similar in appearance Heal in days to weeks with scarringHeal in days to weeks with scarring

Behcet’s DiseaseBehcet’s Disease

Behcets and Churg StraussBehcets and Churg Strauss

CSS has been divided into 3 distinct phases, which may or may not be sequential.

The prodromal phase is characterized by asthma with or without allergic rhinitis.

The second phase is marked by a peripheral blood eosinophilia and eosinophilic tissue infiltration producing a picture similar to Loeffler syndrome, chronic eosinophilic pneumonia, or eosinophilic gastroenteritis.

The third, vasculitic phase may involve any organ. The most frequent site of involvement is the heart. Other organs that may be involved with a vasculitis include the lung, central nervous system, kidney, lymph nodes, muscle, and skin. Skin involvement occurs in more than two-thirds of patients