ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis?
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.
Vaglio
A
A
Department of Clinical Medicine, Nephrology and Health Science, University of Parma, Parma, Italy.
Manenti
L
L
Allegri
L
L
Ferrozzi
F
F
Corradi
D
D
Buzio
C
C
D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic; D018450:Disease Progression; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000473:pathology; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012185:Retroperitoneal Fibrosis / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D014657:Vasculitis / Q000276:immunology*
D019268:Antibodies, Antineutrophil Cytoplasmic