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76) According to the Chapel Hill Consensus Conference on the nomenclature of vasculitides, granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis can be separated based on:

Antineutrophilic cytoplasmic antibody (ANCA) specificity: c-ANCA versus p-ANCA Caliber of the vessels affected by necrosis and inflammation The predominance of arterial versus venous involvement Characteristic pattern of vascular inflammation (granulomatous versus fibrinoid necrosis) Clinical findings (asthma, blood eosinophilia, granulomatous sinusitis)

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• Patients lacking signs of asthma, lung and sinus inflammation, or peripheral blood eosinophilia are best classified as having microscopic polyangiitis.

• Necrotizing inflammatory lesions involving the lungs, nasal sinuses, and kidneys are typically seen in cases of granulomatosis with polyangiitis.

• Peripheral blood eosinophilia and asthma are defining features of eosinophilic granulomatosis with polyangiitis.

• All disease entities are commonly treated aggressively (e.g., with cyclophosphamide).

• Fibrinoid necrosis in all cases of ANCA-associated small vessel vasculitis may evoke a granulomatous inflammatory reaction in the adjacent parenchyma demarcating the area of necrosis. Such a granulomatous inflammatory reaction is most often seen in cases of granulomatosis with polyangiitis; however, it also can be encountered in the setting of eosinophilic granulomatosis with polyangiitis or microscopic polyangiitis.

Jennette JC: Nomenclature and classification of vasculitis: lessons learned from granulomatosis with polyangiitis (Wegener’s granulomatosis). Clin Exp Immunol 2011;164(Suppl 1):7-10.

Tarzi RM, Cook HT, Pusey CD: Crescentic glomerulonephritis: new aspects of pathogenesis. Semin Nephrol 2011;31(4):361-368.

Watts RA, Scott DG: Recent developments in the classification and assessment of vasculitis. Best Pract Res Clin Rheumatol 2009;23(3):429-443.

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Anatomic Pathology: Vascular Pathology

76) According to the Chapel Hill Consensus Conference on the nomenclature of vasculitides, granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis can be separated based on:

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