Spectrum of ANCA-Associated Vasculitis

Excerpt

Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitides (AAV) are severe multisystem diseases characterized by necrotizing inflammation of small blood vessels, few or no immune deposits, and circulating ANCA with specificity chiefly against myeloperoxidase or proteinase-3 [1]. These autoantibodies are believed to be generated following antigenic mimicry by diverse triggers, including viruses (Coxsackie B3, parvovirus B19), silica and drugs (propylthiouracil, allopurinol, hydralazine). ANCA are detected by indirect immunofluorescence microscopy, using patient sera admixed with healthy neutrophils followed by fluorescent staining. The presence of antibodies is confirmed by immunoassay, most often ELISA. Based on histology, type of autoantibodies and organ involvement, three chief forms are recognized: microscopic polyangiitis, granulomatosis with polyangiitis (GPA; Wegener’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) [1]. …