Vasculitis Flashcards by Robbie Gruhl

Basic treatment for all vasculitities

steroids

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Types of large vessel vasculitides

temporal (giant cell)

Takayasu

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Types of medium vessel vasculitis

Polyarteritis nodosa

Kawasaki

Burger

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Small vessel vasculitides

Wegner granulomatosis

microscopic polyangiitis

Chruge-Strauss

Henoch-Schönlein Purpura

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Temporal (giant cell) arteritis

biopsy findings

granulomatous: inflammed vessel wall, giant cells, intimal fibrosis

segmental lesions (need long biopsy)

negative Bx does not exclude disease

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Presentation of temporal arteritis

headache, visual disturbances (opthalmic artery involvement), jaw claudication

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Tx of temporal arteritis

steroids

(Tx if suspected at all! not treating can lead to blindness)

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Takaysu arteritis

which arteries does it normally affect?

aortic arch and branches

classically a young Asian female

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Physical exam findings of Takayasu arteritis

neurologic symptoms

weak or absent upper extremity pulses

(subclavian branches are blocked off)

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Polyarteritis Nodosa

type of vasculitis

medium vessel

necrotizing vasculitis

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Organ involvement of polyarteritis nodosa

anything but the lungs

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Presentation of polyarteritis nodosa

HTN (renal artery involvement)

abdominal pain with melena–dark stool (mesentaric artery involvement)

neurologic distrubances

skin lesions

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What can you find in the serum when you have polyarteritis nodosa?

hepatitis B surface antigen

(HBsAg)

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Bx results of polyarteritis nodosa

“string of pearls”

fibrinoid necrosis (weaking of wall causes aneurysms)

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Tx of polyarteritis nodosa

cyclophosphamide

corticosteroids

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Kawasaki disease

presentation

Asian children < 4 y/o

non-specific Sx: rash on palms and soles, fever, conjuctivitis, enlarged cervical lymph nodes

Biggest complication of Kawasaki disease

coronary artery involvement

thrombosis or aneurysm with rupture

Tx of Kawasaki disease

aspirin

IVIG

Buerger Disease

associated with heavy smoking

autoamputation of digits

Wegner’s granulomatosis

associated serum marker

c-ANCA

cytoplasmic-anti neutrophil cytoplasmic antibody

Microscopic polyangiitis

associated serium marker

p-ANCA

perinuclear-anti-neutrophil cytoplasmic antibody

Churg-Strauss Syndrome

associated serum marker

p-ANCA

Wegner granulomatosis

affected organs

nasopharynx (chronic sinusitis, ulcerations)

lungs (classic: bilateral nodules)

kidneys (renal failure)

Wegner granulomatosis

Bx results

necrotizing granulomas w/ adjacent necrotizing vasculitis

Tx of Wegner's

corticosteroids cyclophosphamide

Microscopic Polyangiitis affected organs

lungs and kidneys

Microscopic Polyangiitis Tx

corticosteroids cyclophosphamide

Differences between Wegner and microscopic polyangiitis

Wegers: **nasopharynx**, lungs, kidneys microscopic polyangiitis: lungs, kidneys Wegners: cANCA microscopic polyangiitis: pANCA

Chrug-Strauss affected organs

lungs and heart

Churg-Strauss associated symptoms

eosinophilia asthma

Chrug-Strauss Bx

necrotizing granulomatous inflammation w/ eosinophils

Henoch-Schönlein Purpura defining pathology

vasculitis due to IgA immune complex deposition type III hypersensitivity

Skin lesions in Henoch-Schönlein

Henoch-Schönlein purpura! palpable purpura raised lesions, usually found on buttocks and legs

What organs does Henoch-Schönlein Purpura normally affect

skin (palpable purpura) kidneys (IgA nephropathy) GI

Tx of Henoch-Schönlein Purpura

resolves on own usually steroids if refractory or recurrent

Most common vasculitis in children

Henoch-Schönlein Purpura

Which vasculitis is associated with p-ANCA

microscopic polyangiitis Churg-Straus

How to differentiate between microscopic polyangiitis and Churg-Strauss

both have p-ANCA microscopic polyangiitis affects lungs and kidneys Churg-Strauss affects heart and lungs Churg-Strauss is has necrotizine granulomatous lesions

How to differentiate between Wegner granulomatosis vs microscopic polyangiitis

Weger: cANCA Microscopic polyangiitis: pANCA Wegner: **granulomatous** lesions of **nasopharynx**, lung, kidney Microscopic polyangiitis: **nongranulomatous** lesions of lung and kidney