Granulomatose de Wegener: a propósito de um caso clínico
Keywords:
ANCA, proptosis, rapidly progressive glomerulonephritis, scleritis, vasculitis, Wegener granulomatosisAbstract
The authors presenta case of Wegener's gra nulomatosis that had as first symptom scleritis, uveitis, and proptosis. Because of rapidly progressive glomerulonephritis, the patient was admitted three months later. On the 12Th day, third of ciclophosphamide therapy, the patient died in respiratory distress. Autopsy disclosed focal and segmentai necrotizing glomerulonephritis with crescent formation, and medium and small vessels necrotizing vasculitis in the lung, liver, and spleen. Necrotizing granulomas, massive alveolar haemorrhage, capillaritis, and extensive fibrosis in the lung was also observed.
Ocular, lung and kidney involvement in the limited and diffuse forms of Wegener's granulomatosis are discussed. A statement on ANCA's value in the diagnosis and follow-up of Wegener's patients is also made.
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