Wegener granulomatosis - an atypical case

An unusual sequence of the clinical manifestations of microvascular disease is described in a 15 year-old girl. She initially presented with acute ren al failure caused by a crescentic glomerulonephritis associated with positi ve tests for MPO-ANCA. Eighteen months later she had pulmonary hemorrhage a nd respiratory failure. An open lune biopsy showed granulomas that were dia gnostic for Wegener granulomatosis. We discuss the diagnostic dilemmas face d in attempts to distinguish infective causes of pulmonary granulomas, such as tuberculosis or fungi, from granulomas associated with vasculitis, in a patient previously treated with immunosuppressive therapy.