Vasculitis

Question 1

How does granulomatosis with polyangiitis present?

Answer 1

Upper respiratory: sinusitis/otitis, saddle-nose deformity
Lower respiratory: Lung nodules/cavitation, hemoptysis
Renal: Rapidly progressive GN
Skin: Livedo reticularis, nonhealing ulcers

Question 2

How is granulomatosis with polyangiitis diagnosed?

Answer 2

Antineutrophil cytoplasmic antibodies (ANCA): PR3 (~70%) and MPO (~20%). c ANCA
Lung biopsy shows granulomatous vasculitis, kidney shows pauci-immune GN (crescents), and skin shows leukocytoclastic vasculitis

Question 3

How is granulomatosis with polyangiitis treated?

Answer 3

With corticosteroids and immunomodulators (e.g. MTX, cyclophosphamide, rituzimab

Question 4

What are risk factors of Takayasu arteritis?

Answer 4

Female, asian, age 10-40

Question 5

What are signs and sx of Takayasu arteritis?

Answer 5

Constitutional (e.g fever, weight loss), arterio-occlusive (e.g. claudication, ulcers) in upper extremities, arthalgias/myalgias. Pts may have BP discrepancies, pulse deficits, and arterial bruits. Pts have elevated ESR and CRP, may have a widened mediastinum on CXR, and artery wall thickening and a narrowed lumen on CT/MRI.

Question 6

What is tx for Takayasu arteritis?

Answer 6

Systemic glucocorticoids

Question 7

What are features of Churg Strauss?

Answer 7

p-ANCA, renal failure, asthma and eosinophilia. Best test is lung bx. Tx w/ cyclophosphamide.

Question 8

What are features of polyarteritis nodosa?

Answer 8

Affects small/med arteries of every organ except the lung! Tx w/ cyclophosphamide. p-ANCA positive, associated with Hep B