ANCA-positive periaortic vasculitis: Does it fall within the spectrum of vasculitis?

Abstract

Vaglio A, Manenti L, Allegri L, Ferrozzi F, Corradi D, Buzio C (University of Parma, Parma, Italy). ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis? (Case Report). J Intern Med 2002; 251: 268-271. Retroperitoneal fibrosis (RPF) is a disease of unknown aetiology that has sometimes been reported in association with connective tissue disorders and systemic vasculitis. We report here two cases of antineutrophil cytoplasmic antibody (ANCA)-positive RPF showing clinical evidence of rapidly progressive glomerulonephritis. Although treatment with pred-nisone and cyclophosphamide led to a remission of RPF in both cases, renal function was restored in only one patient and the other progressed to chronic renal failure. The paper reviews the literature concerning ANCA-positive RPF and discusses the relationship between ANCA-positive vasculitis and RPF.