The history of ANCA-associated vasculitis

An essential early step toward understanding vasculitis was recognition in 1948 of the differences between the small artery disease of polyarteritis, essentially sparing the glomerulus and lungs, and disease of glomerular vessels and small veins, often involving the lungs. By 1951, Churg and Strauss drew on their knowledge of vasculitis literature and renal pathology to provide an authoritative description of the syndrome bearing their names. One year later a paper from Australia described a syndrome of febrile systemic illness with myalgias, arthralgias, microscopic hematuria, and a serum antibody reacting with neutrophil cytoplasm antigens. Within 30 years, nephrologists and immunologists in northern Europe linked antineutrophil cytoplasm antibodies to a specific vasculitis, Wegener's granulomatosis. Falk and Jennette later determined that pANCA reacted with cytoplasmic myeloperoxidase, and that cANCA did not; the antigen with which cANCA reacted was soon identified as a novel serine proteinase. New and better treatments of AAV will follow progress in understanding their pathogenesis.