Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is traditionally viewed as a small vessel disease. We report a patient with cANCA antibodies directed against proteinase-3 with asymptomatic aortic involvement, in combination with diffuse alveolar haemorrhage and pauci-immune, necrotizing crescentic glomerulonephritis. A review of the literature is discussed.
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