Anatomic Pathology: Vascular Pathology

• 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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