Small to Medium Vessels and ANCA

•	Wegener’s granulomatosis (c-ANCA – 96% sensitive)

•	Churg-Strauss syndrome (p-ANCA – 25-70%)

•	microscopic polyangiitis

clinically they can be separated by specific features:

•	Wegener’s – necrotizing granulomatous inflammation in the upper or lower respiratory tract, the kidneys, and occasionally the skin

•	Churg-Strauss – asthma and eosinophilia

•	the absence of specific features leads to the diagnosis of microscopic polyangiitis

Medium Vessel Vasculitis:

•	renal failure occurs much less frequently in CSS compared with others

•	typically lungs not involved in PAN

•	CSS shows significant overlap with WG and PAN

ANCA

Skin:

Systemic:

PAN

+/- pANCA (not helpful)

(50%) cutaneous and subcutaneous nodules +/- ulceration, livedo, esp. LE’s

•	70% - kidneys;

•	lungs spared

•	60% mononeuritis multiplex;

Wegener’s

cANCA sensitive (96%); follow titers

(66%) cutaneous and subcutaneous nodules +/- ulceration, palpable purpura

•

kidneys

•	lung - upper and lower respiratory tract

(necrotizing granulomatous inflammation)

Churg-Strauss

pANCA (25-70%); follow titers

tender nodules on scalp and extremities

•	kidneys spared (usually)

•	lungs - late onset asthma

•	peripheral eosinophilia