proteinase3 (PR-3), seen in Wegeners
myeloperoxidase (MPR), seen in Microscopic polyangitis and Churg-Strauss
not normal in antineutrophil cytoplasmic antibodies. get pulmonary-renal syndromes
Large vessel vascular lesions
Giant Cell Arteritis, Takayasu Arteritis
Medium sized vessel vasc. lesions
polyarteritis nodosa, Kawasaki disease
Small sized vessel vasc. lesions
microscopic polyangitis, Wegener granulomatosis
Giant Cell Arteritis (temporal)
arteries of head, inflm vessel w/ giant cells, breakdown of elastic fibers, fever, headaches, facial pain, vision disturbances
Takayasu Arteritis- Pulseless disease
ocular disturbances, marked weakening of pulses, narrowing of arteries of arch, granulomatous inflammation, unknown cause
transmural necrotizing infl of small/medium arteries, focal random episodic, may produce aneurysmal dilation nodalrity obstruction, varied symptoms, 30% have hep B antigen, little assoc. with ANCA
Kawasaki syndrome (mucocutaneous lymph node syndrome)
very young children, fever oral erythema edema of hands/feet, erythema of palms & soles, viral immun cross rxn, delayed hypersensitivty rxn T cells, up to 20% have heart attacks by age 5
necrotizing vasculitis involving arterioles, capillaries, venules. acute inflm few or no immune deposits (pauci-immune). most have P-ANCA (MPO-ANCA). all lesions same age. involve kidneys, GI, ms, skin, brain etc. clear up by removing offending agent.
microscopic polyangitis (photo and cont)
recruitment and activation of neutrophils give histologic appearance, in some cases clear cut response to antigen drugs (penicillin) microorganisms (streptococci), heterolgous proteins or tumor proteins.
rare small vessel necrot vasc., with asthma allergic rhinitis lung infiltrates perpheral hyperEOSINOPHILA, extravascular necrotizing granulomas, 50% P-ANCA.
renal-respiratory syndromes, most of C-ANCA, focal necrotizing crescentic glomerulonephritis, focal necrotizing vasculities of medium size arteries, upper and lower resp tract, hemoposis.