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 prednisone 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.
ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis? / Vaglio, A; Manenti, L; Allegri, L; Ferrozzi, F; Corradi, D; Buzio, C. - In: JOURNAL OF INTERNAL MEDICINE. - ISSN 0954-6820. - ELETTRONICO. - 251:(2002), pp. ---. [10.1046/j.1365-2796.2002.00948.x]
ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis?
Vaglio, A;
2002
Abstract
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 prednisone 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.
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