ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis?
case_report · Level V
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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.
Medical subject headings
- Antibodies, Antineutrophil Cytoplasmic
- Retroperitoneal Fibrosis
- Vasculitis