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.
Keywords: Wegener’s granulomatosis; aortitis; cANCA; vasculitis.