Vasculitis Overview

VASCULITISVASCULITIS

George R. Mount, CPT USA MCGeorge R. Mount, CPT USA MC

Rheumatology FellowRheumatology Fellow

Walter Reed AMCWalter Reed AMC

GoalsGoals

OverviewOverview Clinical patternsClinical patterns Diagnostic aidsDiagnostic aids Treatment paradigmsTreatment paradigms Cases and questionsCases and questions

IntroductionIntroduction

Inflammation and necrosis of blood Inflammation and necrosis of blood vesselsvessels

Occlusion and ischemiaOcclusion and ischemia Immunologic mechanismsImmunologic mechanisms Multi-system disease with Multi-system disease with

constitutional symptoms and constitutional symptoms and inflammatory laboratory indicesinflammatory laboratory indices

Vasculitis: Primary or Vasculitis: Primary or Secondary?Secondary?

PrimaryPrimary: Vasculitis is the : Vasculitis is the principal principal featurefeature of the disease of the disease

SecondarySecondary:: Vasculitis is a Vasculitis is a complication of another disease or complication of another disease or toxin (e.g. RA, infection, malignancy)toxin (e.g. RA, infection, malignancy)

Classification of VasculitisClassification of Vasculitis

No universally accepted No universally accepted classification systemclassification system– Vessel sizeVessel size– HistopathologyHistopathology– Dominant organ involvementDominant organ involvement

OverlapOverlap

Classification of VasculitisClassification of Vasculitis Large VesselLarge Vessel

– Giant cell arteritisGiant cell arteritis– Takayasu’sTakayasu’s

Medium VesselMedium Vessel– Polyarteritis NodosaPolyarteritis Nodosa– Kawasaki’sKawasaki’s

Small VesselSmall Vessel– Wegener’s, Microscopic polyangitis, Wegener’s, Microscopic polyangitis,

Henoch-Scholein purpura, Cryoglobulemic, Henoch-Scholein purpura, Cryoglobulemic, hypersensitivityhypersensitivity

Classification of VasculitisClassification of Vasculitis

www.rheumtext.com – Hochberg et al (eds)

When to Suspect a VasculitisWhen to Suspect a Vasculitis

Unexplained ischemia:Unexplained ischemia:– Claudication, limb ischemia, angina, Claudication, limb ischemia, angina,

TIA, stroke, mesenteric ischemia, TIA, stroke, mesenteric ischemia, cutaneous ischemiacutaneous ischemia

– Especially in a young individualEspecially in a young individual Multiorgan dysfunction:Multiorgan dysfunction:

– Systemically ill patientSystemically ill patient– Other suggestive featuresOther suggestive features

When to Suspect a VasculitisWhen to Suspect a Vasculitis

Other suggestive features:Other suggestive features:– GlomerulonephritisGlomerulonephritis– Palpable purpuraPalpable purpura– Peripheral neuropathyPeripheral neuropathy– Established autoimmune diseaseEstablished autoimmune disease

When to Suspect a VasculitisWhen to Suspect a Vasculitis

Systemic illness – must exclude Systemic illness – must exclude alternative diagnoses:alternative diagnoses:– SepsisSepsis– Drug toxicityDrug toxicity– MalignancyMalignancy– CoagulopathyCoagulopathy

General Approach to General Approach to DiagnosisDiagnosis

Attempt to exclude other Attempt to exclude other processesprocesses

Consider the age, gender, Consider the age, gender, ethnicity of the patientethnicity of the patient

Determine which organ systems Determine which organ systems are involvedare involved

Estimate the size of the vessels Estimate the size of the vessels involvedinvolved

Demographic AssociationsDemographic Associations

Clues to DiagnosisClues to Diagnosis

Clues to DiagnosisClues to Diagnosis

Clues to DiagnosisClues to Diagnosis

Clues to DiagnosisClues to Diagnosis

Clues to DiagnosisClues to Diagnosis

Approach to Diagnosis: LabsApproach to Diagnosis: Labs

Determine organ involvementDetermine organ involvement Exclude other diseasesExclude other diseases

– Routine labs: CBC, BMP, UA, ESR, CRP, Routine labs: CBC, BMP, UA, ESR, CRP, LFTsLFTs

– Infection w/u: cultures, viral serologies Infection w/u: cultures, viral serologies (HBV, HCV, HIV)(HBV, HCV, HIV)

– Autoimmune serologies: ANA, RF, Autoimmune serologies: ANA, RF, ANCAs, ENA, ds DNA, C3/C4ANCAs, ENA, ds DNA, C3/C4

– Misc: CK, anti-GBM, SPEP, CryoglobulinsMisc: CK, anti-GBM, SPEP, Cryoglobulins

Approach to Diagnosis: Approach to Diagnosis: ANCAs 101ANCAs 101

Antibodies directed against neutrophil Antibodies directed against neutrophil granule constituentsgranule constituents

c-ANCAc-ANCA– Stains cytoplasm (hence “c”)Stains cytoplasm (hence “c”)– Main target antigen: proteinase-3Main target antigen: proteinase-3– Highly specific (>90%) for Wegener’sHighly specific (>90%) for Wegener’s

p-ANCAp-ANCA– Stains perinuclear (hence “p”)Stains perinuclear (hence “p”)– Main target antigen: myeloperoxidaseMain target antigen: myeloperoxidase– A/w MPA and Churg-StraussA/w MPA and Churg-Strauss

Approach to Diagnosis: Approach to Diagnosis: BiopsyBiopsy

Blind biopsy generally low yieldBlind biopsy generally low yield– Less than 20%Less than 20%

““Go where the money is.”Go where the money is.”– Approx. 66% success in symptomatic organsApprox. 66% success in symptomatic organs– Examples: Temporal artery biopsy, kidney Examples: Temporal artery biopsy, kidney

biopsy, sural nerve biopsy, testicular biopsybiopsy, sural nerve biopsy, testicular biopsy Vasculitic lesions tend to be focal and Vasculitic lesions tend to be focal and

segmentalsegmental

Approach to Diagnosis: Approach to Diagnosis: AngiogramAngiogram

If biopsy is impracticalIf biopsy is impractical Important in large vessel vasculitisImportant in large vessel vasculitis Patient with abdominal painPatient with abdominal pain

– Renal or mesenteric vasculitisRenal or mesenteric vasculitis

Approach to Diagnosis: Approach to Diagnosis: AngiogramAngiogram

PrognosisPrognosis

Untreated is a rapidly progressive, Untreated is a rapidly progressive, usually fatal, diseaseusually fatal, disease

The prognosis is determined by the The prognosis is determined by the extent and number of organs extent and number of organs involved involved

Morbidity and mortality can be Morbidity and mortality can be prevented if recognized and prevented if recognized and treated earlytreated early

TreatmentTreatment Determined by:Determined by:

– Type and severity of organ involvementType and severity of organ involvement– Rate of disease progression Rate of disease progression

CorticosteroidsCorticosteroids– Usually 1mg/kg initiallyUsually 1mg/kg initially– Pulse dose corticosteroidsPulse dose corticosteroids

Immunosuppressive therapyImmunosuppressive therapy– Cytoxan, Methotrexate, Imuran Cytoxan, Methotrexate, Imuran

CaseCase A previously healthy 22yo male college

student had an URI 2 weeks ago, RX with PCN

He develops abdominal pain, bilateral ankle pain & swelling with raised purpuric lesions over lower extremities

Labs:– creatinine 3.0 mg/dL, BUN 46 mg/dL– Urinalysis: 4+ proteinuria, 2+ RBC’s, sev. RBC

casts/ hpf

CaseCase

CaseCase What is the most likely cause of renal

disease in this patient:A. Lupus nephritisB. Acute post-streptococcal glomerulonephritisC. Henoch-Schonlein purpuraD. Allergic interstitial nephritisE. Goodpasture’s syndrome

CaseCase What is the most likely cause of renal

disease in this patient:A. Lupus nephritisB. Acute post-streptococcal glomerulonephritisC. Henoch-Schonlein purpuraD. Allergic interstitial nephritisE. Goodpasture’s syndrome

Henoch-Schonlein PurpuraHenoch-Schonlein Purpura

Small vessels, post capillary venules Small vessels, post capillary venules Palpable purpura, arthralgias, Palpable purpura, arthralgias,

abdominal pain, renal diseaseabdominal pain, renal disease Males=femalesMales=females Mean age 5 yrs.Mean age 5 yrs. Preceding URI in 2/3 (1-3 weeks)Preceding URI in 2/3 (1-3 weeks) Tissue deposition of Tissue deposition of IgA-containing -containing

immune complexes (skin, kidneys, (skin, kidneys, bowel)bowel)

Henoch-Schonlein PurpuraHenoch-Schonlein Purpura

GI involvement in 85%GI involvement in 85%– Severe cramping, pain, nausea, Severe cramping, pain, nausea,

vomiting, bleedingvomiting, bleeding– Major hemorrage or intussuseption Major hemorrage or intussuseption

is an uncommon but life-threatening is an uncommon but life-threatening complication in childrencomplication in children

Henoch-Schonlein PurpuraHenoch-Schonlein Purpura

Renal involvement (10-50%)Renal involvement (10-50%)– Renal disease more severe in adultsRenal disease more severe in adults– Determines prognosisDetermines prognosis– Many recover with no therapyMany recover with no therapy– Asymptomatic hematuria Asymptomatic hematuria

proteinuria & renal insufficiency proteinuria & renal insufficiency (cresentic GN)(cresentic GN)

– < 0.5% progress to ESRD< 0.5% progress to ESRD

Henoch-Schonlein PurpuraHenoch-Schonlein Purpura

UncommonUncommon– Testicular involvementTesticular involvement– Pulmonary hemorrhagePulmonary hemorrhage– CNS complicationsCNS complications

Henoch-Schonlein PurpuraHenoch-Schonlein Purpura

Usually single episodes < 4 weeks Usually single episodes < 4 weeks durationduration

40% recurrence rate after period of 40% recurrence rate after period of wellnesswellness

TreatmentTreatment– Supportive measuresSupportive measures– Corticosteroids for GI vasculitis and Corticosteroids for GI vasculitis and

hemorrhagehemorrhage– ? CS early in nephritis? CS early in nephritis

CaseCase 50yo woman presents with 1 week of fever,

chills, chest pain, cough, dyspnea and paresthesias in LE

PMHx: bronchial asthma (7 yrs), allergic rhinitis

PE: 100 F, HR 98, BP 120/70, RR 16/min– wheezes/ rhonchi bilaterally– mild peripheral weakness in LE– sensory dysesthesia in stocking distribution

bilaterally

CaseCase Lab: WBC 12.8 (N 30%, L 25%, Eos Lab: WBC 12.8 (N 30%, L 25%, Eos

40%)40%) Blood/sputum cultures: negativeBlood/sputum cultures: negative ANA neg, p-ANCA + 1:20ANA neg, p-ANCA + 1:20 CXR: patchy bilateral infiltratesCXR: patchy bilateral infiltrates

CaseCase

CaseCaseShe is treated with empiric antibiotics over 3 days with no improvement in symptoms.

What is the most likely diagnosis:A. Eosinophilic bronchitisB. Idiopathic hypereosinophilic syndromesC. Churg-Strauss syndromeD. Wegener’s granulomatosisE. Polyarteritis nodosa

CaseCaseShe is treated with empiric antibiotics over 3 days with no improvement in symptoms.

What is the most likely diagnosis:A. Eosinophilic bronchitisB. Idiopathic hypereosinophilic syndromesC. Churg-Strauss syndromeD. Wegener’s granulomatosisE. Polyarteritis nodosa

Churg-Strauss VasculitisChurg-Strauss Vasculitis

Necrotizing, granulomatous vasculitis of Necrotizing, granulomatous vasculitis of small arteries and venulessmall arteries and venules

Prior asthmaPrior asthma– Started on leukotriene inhibitors Started on leukotriene inhibitors

and weaned off steroidsand weaned off steroids Allergic rhinitisAllergic rhinitis EosinophiliaEosinophilia Pulmonary infiltratesPulmonary infiltrates Intra/extravascular granulomasIntra/extravascular granulomas

Churg-Strauss: Criteria- 4/6Churg-Strauss: Criteria- 4/6

AsthmaAsthma Eosinophilia (>10%)Eosinophilia (>10%) Mono/ PolyneuropathyMono/ Polyneuropathy Pulmonary Infiltrates – Non-fixedPulmonary Infiltrates – Non-fixed Paranasal sinus abnormalityParanasal sinus abnormality Extravascular eosinophilsExtravascular eosinophils

Churg-Strauss VasculitisChurg-Strauss Vasculitis

Asthma precedes vasculitisAsthma precedes vasculitis Confusion with Wegener’sConfusion with Wegener’s

– Nasal/sinus DZ is NON-destructiveNasal/sinus DZ is NON-destructive– Pulmonary nodules less commonPulmonary nodules less common

Churg-Strauss VasculitisChurg-Strauss Vasculitis

p-ANCA (MPO): 70%p-ANCA (MPO): 70% More responsive to steroids aloneMore responsive to steroids alone

CaseCase

A 65yo woman c/o 6 months of malaise, 9lb A 65yo woman c/o 6 months of malaise, 9lb weight loss, recurrent sinusitis, and a weight loss, recurrent sinusitis, and a persistent cough. On exam, she is afebrile, persistent cough. On exam, she is afebrile, the mid-portion of the nasal bridge has a the mid-portion of the nasal bridge has a flattened appearance, and both sides of the flattened appearance, and both sides of the nasal septum are ulcerated. RF is positive nasal septum are ulcerated. RF is positive and ESR is 66.and ESR is 66.

Which of the following tests would be most Which of the following tests would be most helpful in determining the diagnosis:helpful in determining the diagnosis:A. Nasal septum biopsyA. Nasal septum biopsyB. Chest radiographB. Chest radiographC. Measurement of ANCA antibodiesC. Measurement of ANCA antibodiesD. Sputum cultureD. Sputum cultureE. Measurement of anti-GBM antibodiesE. Measurement of anti-GBM antibodies

CaseCase

A 65yo woman c/o 6 months of malaise, 9lb A 65yo woman c/o 6 months of malaise, 9lb weight loss, recurrent sinusitis, and a weight loss, recurrent sinusitis, and a persistent cough. On exam, she is afebrile, persistent cough. On exam, she is afebrile, the mid-portion of the nasal bridge has a the mid-portion of the nasal bridge has a flattened appearance, and both sides of the flattened appearance, and both sides of the nasal septum are ulcerated. RF is positive nasal septum are ulcerated. RF is positive and ESR is 66.and ESR is 66.

Which of the following tests would be most Which of the following tests would be most helpful in determining the diagnosis:helpful in determining the diagnosis:A. Nasal septum biopsyA. Nasal septum biopsyB. Chest radiographB. Chest radiographC. Measurement of ANCA antibodiesC. Measurement of ANCA antibodiesD. Sputum cultureD. Sputum cultureE. Measurement of anti-GBM antibodiesE. Measurement of anti-GBM antibodies

Wegener’s GranulomatosisWegener’s Granulomatosis

Necrotizing, granulomatous vasculitis Necrotizing, granulomatous vasculitis small vesselssmall vessels

Affects persons of any ageAffects persons of any age No significant sex predilectionNo significant sex predilection Upper and lower respiratory tractsUpper and lower respiratory tracts Glomerulonephritis Glomerulonephritis Frequently vasculitis of other organsFrequently vasculitis of other organs

Wegener’s GranulomatosisWegener’s Granulomatosis

Nasal/ sinus disease destructiveNasal/ sinus disease destructive Renal follows respiratoryRenal follows respiratory

– May progress rapidlyMay progress rapidly Non-specific abnormalitiesNon-specific abnormalities

– Conjunctivitis, scleritis, episcleritisConjunctivitis, scleritis, episcleritis– Proptosis (15%)Proptosis (15%)

Wegener’s GranulomatosisWegener’s Granulomatosis

Criteria: 2 or moreCriteria: 2 or more– Nasal/oral ulcers OR purulent/ Nasal/oral ulcers OR purulent/

bloody dischargebloody discharge– Abnormal CXR - nodules, focal Abnormal CXR - nodules, focal

infiltrates, cavitiesinfiltrates, cavities– Abnormal urine sediment Abnormal urine sediment

(microhematuria, RBC casts)(microhematuria, RBC casts)– Granulomatous inflammationGranulomatous inflammation

Wegener’s GranulomatosisWegener’s Granulomatosis

c-ANCAc-ANCA– > 90% + in patients with classic symptoms> 90% + in patients with classic symptoms– Facilitates clinical DXFacilitates clinical DX– Does not eliminate need for BXDoes not eliminate need for BX– Not for intensification of therapyNot for intensification of therapy

Open biopsyOpen biopsy– Paranasal, nasal, larynx, lungParanasal, nasal, larynx, lung– Renal biopsy rarely distinctive enough to Renal biopsy rarely distinctive enough to

be definitivebe definitive

Wegener’s GranulomatosisWegener’s Granulomatosis

Outcomes:Outcomes:

Intervention Survival

None 50% at 5 months

Glucocorticoids 50% at 1 year

GCS + Cytoxan 80% at 8 years

Distinguishing CSV & WGDistinguishing CSV & WG

CSV WG

Asthma +++ uncommon

Eosinophils +++ occ /modest

Atopy +++ uncommon

Upp airway destruction uncommon +

Pulmonary nodules occasional ++

Renal failure + ++

CaseCase A 29yo woman presents with a 4yr h/o skin ulcers

on her lower extremities. A previous punch biopsy showed thrombotic lesions in small blood vessels of the dermis. Treatment has mostly focused on wound care. On exam the ulcers are noted, as is livedo reticularis, a decreased right hand grip, and a right foot drop.

Which of the following is the most likely diagnosis:A. Lymphoma, with a paraneoplastic syndromeB. Takayasu’s arteritisC. Systemic lupus erythematosusD. Polyarteritis nodosaE. Kawasaki’s disease

CaseCase A 29yo woman presents with a 4yr h/o skin ulcers

on her lower extremities. A previous punch biopsy showed thrombotic lesions in small blood vessels of the dermis. Treatment has mostly focused on wound care. On exam the ulcers are noted, as is livedo reticularis, a decreased right hand grip, and a right foot drop.

Which of the following is the most likely diagnosis:A. Lymphoma, with a paraneoplastic syndromeB. Takayasu’s arteritisC. Systemic lupus erythematosusD. Polyarteritis nodosaE. Kawasaki’s disease

Polyarteritis Nodosa Polyarteritis Nodosa

Primary systemic necrotizing Primary systemic necrotizing vasculitisvasculitis– Small/ medium sized arteriesSmall/ medium sized arteries– Very rarely veinsVery rarely veins– Never large elastic arteriesNever large elastic arteries– May be a manifestation of other diseaseMay be a manifestation of other disease

RA, Sjogren’s, Hepatitis B or CRA, Sjogren’s, Hepatitis B or C

– Limited Limited progressive/ fulminant progressive/ fulminant

Polyarteritis NodosaPolyarteritis Nodosa

Any ageAny age Peak years: 30-60Peak years: 30-60 M:F Ratio 2:1M:F Ratio 2:1

Fever, malaise, weight lossFever, malaise, weight loss Arthritis/ arthralgia (50%)Arthritis/ arthralgia (50%) Skin lesions Skin lesions Neurologic (Peripheral > central) (50-70%)Neurologic (Peripheral > central) (50-70%) Renal (70%)Renal (70%) HTN (25%)HTN (25%) Cardiac (50%)Cardiac (50%) GI (50%)GI (50%) ““Classic PAN”- Rare lung diseaseClassic PAN”- Rare lung disease

Polyarteritis NodosaPolyarteritis Nodosa

Anemia, leukocytosis, thrombocytosisAnemia, leukocytosis, thrombocytosis ESR elevationESR elevation Hypocomplementemia (25%)Hypocomplementemia (25%) Hepatitis B SAg (10-54%)Hepatitis B SAg (10-54%) Hep C Ab: 5%Hep C Ab: 5% p-ANCA (MPO): < 10%, c-ANCA is rarep-ANCA (MPO): < 10%, c-ANCA is rare

Polyarteritis NodosaPolyarteritis Nodosa

PAN-DiagnosisPAN-Diagnosis

Biopsy – Symptomatic sitesBiopsy – Symptomatic sites– Skin, Sural nerve, Muscle, Liver, Testes, Skin, Sural nerve, Muscle, Liver, Testes,

Temporal ArteryTemporal Artery– Renal- does not allow differentiation of Renal- does not allow differentiation of

type of vasculitis (segmental necrotizing type of vasculitis (segmental necrotizing GN)GN)

ANGIO - ABDOMINAL VISCERA ANGIO - ABDOMINAL VISCERA – Evidence of intra-abdominal involvementEvidence of intra-abdominal involvement– Other involved organs not available for bxOther involved organs not available for bx

PAN- Prognosis &TreatmentPAN- Prognosis &Treatment

Untreated - 85% mortality at 5 Untreated - 85% mortality at 5 yearsyears

Treatment - 80% survival at 5 Treatment - 80% survival at 5 yearsyears– CorticosteroidsCorticosteroids– CytotoxicsCytotoxics

40% relapse (median 33 months)40% relapse (median 33 months)

CaseCase

A 32yo Korean woman presents with a 30lb A 32yo Korean woman presents with a 30lb weight loss, low-grade fevers and arthralgias. weight loss, low-grade fevers and arthralgias. She notes back pain between her shoulder She notes back pain between her shoulder blades. She notes pain in her arms with any blades. She notes pain in her arms with any prolonged activity. Recent labs are notable for prolonged activity. Recent labs are notable for a platelet count of 800K and an ESR of 130. On a platelet count of 800K and an ESR of 130. On exam, her HR is 100bpm and her BP is 60/40 in exam, her HR is 100bpm and her BP is 60/40 in both arms.both arms.

What is the next step in her management:What is the next step in her management:A.A. Hospitalize for further evaluationHospitalize for further evaluationB.B. Order blood cultures, get an ANA and RFOrder blood cultures, get an ANA and RFC.C. Perform a careful exam, listen for subclavian bruitsPerform a careful exam, listen for subclavian bruitsD.D. Administer IV salineAdminister IV salineE.E. Advise the patient to begin Fe supplementationAdvise the patient to begin Fe supplementation

CaseCase

A 32yo Korean woman presents with a 30lb A 32yo Korean woman presents with a 30lb weight loss, low-grade fevers and arthralgias. weight loss, low-grade fevers and arthralgias. She notes back pain between her shoulder She notes back pain between her shoulder blades. She notes pain in her arms with any blades. She notes pain in her arms with any prolonged activity. Recent labs are notable for prolonged activity. Recent labs are notable for a platelet count of 800K and an ESR of 130. On a platelet count of 800K and an ESR of 130. On exam, her HR is 100bpm and her BP is 60/40 in exam, her HR is 100bpm and her BP is 60/40 in both arms.both arms.

What is the next step in her management:What is the next step in her management:A.A. Hospitalize for further evaluationHospitalize for further evaluationB.B. Order blood cultures, get an ANA and RFOrder blood cultures, get an ANA and RFC.C. Perform a careful exam, listen for subclavian bruitsPerform a careful exam, listen for subclavian bruitsD.D. Administer IV salineAdminister IV salineE.E. Advise the patient to begin Fe supplementationAdvise the patient to begin Fe supplementation

Takayasu’s ArteritisTakayasu’s Arteritis

Large vesselLarge vessel Unknown etiologyUnknown etiology Aorta/branchesAorta/branches ““Pulseless Disease”Pulseless Disease”

Takayasu’s ArteritisTakayasu’s Arteritis

Women in reproductive yearsWomen in reproductive years– 10X more than men10X more than men– Asia, Eastern Europe, Latin AmericaAsia, Eastern Europe, Latin America

Granulomatous PanarteritisGranulomatous Panarteritis

Takayasu’s ArteritisTakayasu’s Arteritis

98% have stenotic lesions, 27% 98% have stenotic lesions, 27% aneurysmsaneurysms

Subclavian & aortic arch most Subclavian & aortic arch most common, 93%common, 93%

40- 80% renal artery stenosis40- 80% renal artery stenosis

Arterial stenoses/organ ischemiaArterial stenoses/organ ischemia– ClaudicationClaudication– Transient cerebral ischemia/ strokeTransient cerebral ischemia/ stroke– Renal artery hypertensionRenal artery hypertension– CHFCHF– AnginaAngina– MIMI– Mesenteric vascular insufficiencyMesenteric vascular insufficiency

Takayasu’s ArteritisTakayasu’s Arteritis

In the absence of complications In the absence of complications (retinopathy, HTN, aortic v. insuff), (retinopathy, HTN, aortic v. insuff), 15 yr survival 95%15 yr survival 95%

Most respond to steroids aloneMost respond to steroids alone 40% will need cytotoxics40% will need cytotoxics

Takayasu’s ArteritisTakayasu’s Arteritis

CaseCase A 78yo woman c/o headache for the A 78yo woman c/o headache for the

past 8 days and notes the onset of past 8 days and notes the onset of double vision and blurring, lasting double vision and blurring, lasting 15min before resolving. She has lost 15min before resolving. She has lost 15lb over the past 2 months.15lb over the past 2 months.

Which is the best next step in her Which is the best next step in her management:management:

A.A. Refer her to an ophthalmologist or neuroRefer her to an ophthalmologist or neuroB.B. Schedule a temporal artery biopsySchedule a temporal artery biopsyC.C. Test her ESR and schedule a TA biopsyTest her ESR and schedule a TA biopsyD.D. Administer sumitriptanAdminister sumitriptanE.E. Administer prednisone, 60mg, Administer prednisone, 60mg,

immediately and schedule a TA biopsyimmediately and schedule a TA biopsy

CaseCase A 78yo woman c/o headache for the A 78yo woman c/o headache for the

past 8 days and notes the onset of past 8 days and notes the onset of double vision and blurring, lasting double vision and blurring, lasting 15min before resolving. She has lost 15min before resolving. She has lost 15lb over the past 2 months.15lb over the past 2 months.

Which is the best next step in her Which is the best next step in her management:management:

A.A. Refer her to an ophthalmologist or neuroRefer her to an ophthalmologist or neuroB.B. Schedule a temporal artery biopsySchedule a temporal artery biopsyC.C. Test her ESR and schedule a TA biopsyTest her ESR and schedule a TA biopsyD.D. Administer sumitriptanAdminister sumitriptanE.E. Administer prednisone, 60mg, Administer prednisone, 60mg,

immediately and schedule a TA biopsyimmediately and schedule a TA biopsy

Giant Cell ArteritisGiant Cell Arteritis

““Temporal arteritis” & “Cranial arteritis”Temporal arteritis” & “Cranial arteritis” Large vessel, granulomatous arteritisLarge vessel, granulomatous arteritis Extracranial vessels (arteries) >>> Extracranial vessels (arteries) >>>

intracranialintracranial Aortic arch vessels (10-15%)Aortic arch vessels (10-15%) Unknown etiologyUnknown etiology Persons over age 50 (mean 70)Persons over age 50 (mean 70) 2-3x more common in women2-3x more common in women

Segmental vessel inflammationSegmental vessel inflammation Multinucleate giant cell, lymphocytic Multinucleate giant cell, lymphocytic

predominance…PMN’s rarepredominance…PMN’s rare ThrombosisThrombosis Giant cells not Giant cells not required required for diagnosisfor diagnosis

Giant Cell ArteritisGiant Cell Arteritis

Nonspecific constitutional symptoms- fever,malaise, Nonspecific constitutional symptoms- fever,malaise, fatiguefatigue

Headache (> 2/3)Headache (> 2/3) Scalp tenderness +/- nodulesScalp tenderness +/- nodules Temporal artery tendernessTemporal artery tenderness Visual symptoms (blindness 15%)Visual symptoms (blindness 15%) Intermittent claudication (jaw, tongue, extremities)Intermittent claudication (jaw, tongue, extremities) Neuropathies/TIA/Stroke (30%)Neuropathies/TIA/Stroke (30%) Respiratory Tract (10%)Respiratory Tract (10%) PMR (40-60%)PMR (40-60%)

Giant Cell ArteritisGiant Cell Arteritis

Giant Cell ArteritisGiant Cell Arteritis LabLab

– ESR – Marked elevationESR – Marked elevation– CRP elevationCRP elevation– AnemiaAnemia– ThrombocytosisThrombocytosis– Increased LAEIncreased LAE– Increased Alkaline phosphotaseIncreased Alkaline phosphotase

BiopsyBiopsy– Vascular involvement not uniformVascular involvement not uniform– Length ?: If normal TA exam, obtain 3-Length ?: If normal TA exam, obtain 3-

5 cm sample and examine at multiple 5 cm sample and examine at multiple levelslevels

– Negative biopsy Negative biopsy – Consider contralateral bx if first bx Consider contralateral bx if first bx

normalnormal– When ? .….ASAPWhen ? .….ASAP

Giant Cell ArteritisGiant Cell Arteritis

Prednisone 1mg/kg/dPrednisone 1mg/kg/d– Taper 10%/month after Sx/lab Taper 10%/month after Sx/lab

resolvedresolved– Slow taper at 15 mg/dSlow taper at 15 mg/d– Lab and SxLab and Sx– Long term steroidsLong term steroids

Steroid sparing with MTXSteroid sparing with MTX

Giant Cell ArteritisGiant Cell Arteritis

30% - relapse with steroid taper, esp 30% - relapse with steroid taper, esp prednisone < 20 mg/dprednisone < 20 mg/d

25% - vertebral compression fractures25% - vertebral compression fractures 50% - other serious steroid toxicities, 50% - other serious steroid toxicities,

e.g. HTN, diabetes, CHF, cataracts…..e.g. HTN, diabetes, CHF, cataracts…..

Giant Cell ArteritisGiant Cell Arteritis

Common Vaculitis Presentations Common Vaculitis Presentations (aka Board Buzz Words…)(aka Board Buzz Words…)

Oral & genital ulcers = Behcet’sOral & genital ulcers = Behcet’s Upper/lower airway disease and Upper/lower airway disease and

glomerulonephritis = Wegener’sglomerulonephritis = Wegener’s– Septal perforation, epistaxisSeptal perforation, epistaxis– Recurrent sinus infectionsRecurrent sinus infections

Young female with arm/leg Young female with arm/leg fatigue and HTN = Takayasu’sfatigue and HTN = Takayasu’s

Common Vaculitis Presentations Common Vaculitis Presentations (aka Board Buzz Words…)(aka Board Buzz Words…)

Palpable purpura = small Palpable purpura = small vessel leukocytoclastic vessel leukocytoclastic vasculitisvasculitis

Hepatitis B = PANHepatitis B = PAN Hepatitis C = cryoglobulinemiaHepatitis C = cryoglobulinemia

Questions?