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Although C-ANCA and P-ANCA are typically of the IgG isotype, ANCA restricted to the IgM isotype are associated with pulmonary hemorrhage, as are ANCA/MPO antibodies in about 30% of glomerular basement membrane disease. Eighty-eight percent of patients with vasculitis-associated pulmonary hemorrhage have elevated IgM ANCA; whereas, no patients who are IgM ANCA-negative have pulmonary hemorrhage. IgA ANCA is associated with Cholangitis, Kawasaki disease, Cystic fibrosis and Henoch-Schonlein purpura.
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