Vasculitides

Question 1

Temporal (giant cell) arteritis

Answer 1

• Older women (but not always)
• ESR elevated
• Jaw claudication
• May lead to blindness (ophthalmic a. occlusion)
• Associated w/ Polymyalgia Rheumatica
• Focal granulomatous inflammation (branches of carotid a.)
• Mediated by IL-6

Question 2

Takayasu arteritis

Answer 2

“Temporal arteritis of the aorta”

• Affects aorta and branches (obviously)
• Granulomatous (obviously)
• ESR elevated (obviously)
• Young (asian) women
• “Pulseless disease”

Question 3

Buerger’s disease (thromboangiitis obliterans)

Answer 3

• Young male smokers
• Gangrene/autoamputation of digits
• Raynaud’s
• Segmental thrombosing vasculitis, also affecting veins + nerves

Question 4

Kawasaki disease

Answer 4

CRASH & burn:

• Conjunctiva
• Rash
• Adenopathy
• Strawberry tongue
• Hand-foot changes
• Fever (“burn”)
• Coronary artery aneurysms
• Treat with ASA (one of the few diseases you do this with b/c Reye’s syndrome)

Question 5

Polyarteritis Nodosa

Answer 5

• Associated w/ Hep B
• Innumerable microaneurysms (renal “beads on a string,” but can occur elsewhere e.g. GI but not lung)
• Fibrinoid necrosis w/ transmural inflammation
• Nonspecific sx (malaise, weight loss, headache)
• GI (mesenteric ischemia, melena, abd pain)
• Mononeuritis multiplex

Question 6

Behcet syndrome

Answer 6

• Turkish/Mediterranean
• Aphthous/genital ulcers + uveitis
• Erythema nodosum
• Associated w/ HSV, parvovirus
• HLAB51
• Immune complex vasculitis

Question 7

Cutaneous small vessel vasculitis

Answer 7

• Palpable purpura only, 7-10 days after meds (pencillins, cephalosporins, phenytoin, allopurinol), infections (HCV, HIV)
• Immune complex leukocytoclastic vasculitis; systemic involvement later

Question 8

EGPA (Churg-Strauss)

Answer 8

GPA plus:

• Heart, GI, skin (nodules or purpura), neuropathy (e.g. wrist/foot drop)
• Eosinophilia, asthma
• p-ANCA (instead of c-ANCA)

Question 9

GPA (Wegner’s)

Answer 9

The “c” mnemonic:

• c-ANCA (= PR3-ANCA)
• c-shaped saddle nose
• C through the body - hits lungs, kidney, nose
• Cavitary lesions in the lung (necrotizing granulomas)
• Cyclophosphamides, Corticosteroids (treatment)
• Crescentic glomerulonephritis (possible sequelae; Pauci-immune i.e. negative IF)

Question 10

Henoch-Schonlein Purpura (IgA vasculitis)

Answer 10

• Triad: GI (abd pain), buttock/leg purpura, arthralgias
• IgA deposition (type III HSR; systemic version of Berger disease - IgA nephropathy)
• Perivascular neutophilic infiltrate (a “leukocytoclastic” vasculitis)
• Follows URI

Question 11

Microscopic polyangiitis

Answer 11

GPA w/o nasopharyngeal involvement (or granulomas):

• No granulomas
• Lung, kidney, skin (purpura)
• p-ANCA (instead of c-ANCA)
• Crescentic glomerulonephritis (pauci-immune = negative IF)

Question 12

Mixed cryoglobulinemia

Answer 12

• HCV, etc (associated with other viruses)
• Triad: Palpable purpura, weakness, arthralgias
• Plus peripheral neuropathy, renal disease
• “Mixed” IgG, IgM deposits
• Cryoglobulins precipitate in the cold (duh)