IgA nephropathy is the commonest cause of glomerulonephritis worldwide, and is usually a renal-limited disease. In rare cases, IgA nephropathy may also present with a pulmonary–renal syndrome in which pulmonary hemorrhage is a critical feature. Patients presenting with IgA nephropathy and pulmonary hemorrhage have high morbidity and are at high risk for mortality unless rapid immunosuppressive therapy is instituted. We present a case of IgA nephropathy complicated by pulmonary hemorrhage in which immunosuppressive therapy led to a good outcome, and review the literature on similar cases and the outcome of therapy.

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