Vasculitides (STEP 1 Book) Flashcards by Joseph Calderaro

What are the large vessel vasculitis (2)?

Temporal (giant cell) arteritis

Takayasu arteritis

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Temporal arteritis presents as?

Unilateral headache, jaw claudication
Irreversible blindness due to ophthalmic artery occlusion
Polymyalgia rheumatica association

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Temporal arteritis most commonly goes after which artery?

Carotid artery

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What type of inflammation involved in Temporal arteritis?

Focal granulomatous inflammation with increase in ESR

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How do you treat Temporal Arteritis?

High dose corticosteroids prior to artery biospy

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Takayasu arteritis characterized by?

“Pulseless disease” (Weak upper extremity pulses)
Ocular disturbances
Fever
Skin nodules

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Lab findings of Takayasu arteritis?

Granulomatous thickening and narrowing of aortic arch and proximal great vessels
Increased ESR

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How do you treat Takayasu?

Corticosteroids

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What are the medium vessel vasculitis (3)?

Polyarteritis nodosa
Kawasaski disease
Berger disease (Thromboangiitis obliterates)

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Who does Polyarteritis nodosa occur in?

Young adults

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What does Hepatitis B have to do with Polyarteritis Nododa?

30% of patients have Hep b seropositivity

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What vessels does Polyarteritis nodosa affect?

Renal and visceral vessels, no pulmonary arteries

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What causes the vasculitis in Polyarteritis nodosa?

Immune complex mediated

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What type of necrosis in Polyarteritis nodosa?

Fibrinoid necrosis of arterial wall

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What affect does Polarteritis nodosa have on the kidney?

Innumerable renal micro aneurysms and spasms

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How do you treat Polyarteritis nodosa?

Corticosteroids

Cyclophosphamide

Who does Kawasaki disease occur in?

Asian children less than 4 years old

CRASH and Burn mnemonic for Kawasaki?

Conjunctival injection
Rash
Adenopathy
Strawberry Tongue
Hand foot changes
Burn = fever

Cause of death for Kawasaki cases?

Coronary artery aneurysms thrombosis or rupture

How do you treat Kawasaski

IV immunoglobulin

Aspirin

Who is Buerger disease in?

Heavy smokers less than 40

Danger of Buerger disease?

Intermittent claudication leads to gangrene
Autoamputation of digits
Superficial nodular phlebitis
Raynauds

How do you treat Buerger disease?

Have them stop smoking

What are the small vessel vasculitis (4)?

Granulomatosis with polyangitis (Wegener)
Microscopic polyangitis
Eosinophilic granulomatosis with polyangitis (Churg-Strauss)
Henoch-Schonlein purpura

What is Wegener vasculitis triad?

Focal necrotizing vasculitis Necrotizing granulomas in lung/upper airway Necrotizing glomerulonephritis

Wegener's associated with what antibody?

Anti-proteinase 3 | c-ANCA

Treatment for Wegener's?

Cyclophosphamide | Corticosteroids

What is Microscopic Polyangitis?

Necrotizing vasculitis commonly involving lungs, kidneys, and skin with glomerulonephritis and palpable purpura

Microscopic is similar to Wegener's minus what feature?

Nasopharyngeal involvement, Microscopic has none | No granulomas

Antibod associated with Microscopic polyangitis?

Anti-myeloperoxidase | p-ANCA

How do you treat Microscopic polyangitis?

Cyclophosphamide | Corticosteroids

What does Eosinophilic granulomatosis with polyangitis cause?

Asthma, sinusitis, skin nodules, peripheral neuropathy | Heart, GI, kidneys (glomerulonephritis)

How does Eosinophillic granulomatosis present?

Granulomatous necrotizing vasculitis with eosinophilia

What antibody does Eosinophilic granulomatosis associate with?

p-ANCA | Increase in IgE

Henoch Schonlein is what?

Most common childhood systemic vasculitis that follows URI

Classic triad of Henoch Schonlein?

Palpable purpura on ass/legs Arthralgia GI abdominal pain

What causes Henoch Schonlein?

Vasculitis secondary to IgA immune complex deposition

What other disease does Henoch schonlein associate with?

Berger disease (IgA neuropathy)

Easy way to remember treatments for vasculitis?

Only one that isn't corticosteroids is Kawasaki which is immunoglobulin and aspirin