Predictive value of initial histology and effect of plasmapheresis on long-term prognosis of rapidly progressive glomerulonephritis

Zäuner, Ingeborg and Bach, Dieter and Braun, Norbert and Kraemer, Bernhard K. and Fuenfstueck, Reinhard and Helmchen, Udo and Schollmeyer, Peter and Boehler, Joachim (2002) Predictive value of initial histology and effect of plasmapheresis on long-term prognosis of rapidly progressive glomerulonephritis. AMERICAN JOURNAL OF KIDNEY DISEASES, 39 (1). pp. 28-35. ISSN 0272-6386

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Abstract

Intensive immunosuppressive therapy has improved the outcome of patients with rapidly progressive glomerulonephritis (RPGN), which progresses to end-stage renal failure in 90% of patients without intervention. However, it remains unclear which patients benefit most from immunosuppressive therapy and whether plasmapheresis improves long-term outcome. This prospective multicenter study randomized 39 patients with biopsy-proven RPGN (Couser type II, n = 6; pauci-immune type III, n = 33) to undergo either immunosuppressive therapy with prednisone and cyclophosphamide (n = 18) or plasmapheresis in addition to immunosuppression (n = 21). Patients were observed for a mean of 127 months or until reaching the end points of hemodialysis or death. Six of 11 patients who were initially dialysis dependent recovered renal function; however, 2 of those patients required dialysis therapy again after 10 and 105 months. Overall, 15 of 39 patients reached end-stage renal failure after a mean of 25 months, and 4 patients died before requiring hemodialysis therapy. Plasmapheresis had no significant effect on renal or patient survival in type 11 or pauci-immune (type 111) RPGN, independently of age, sex, or serum creatinine level at the time of diagnosis. Overall, probabilities of dialysis-free survival were 0.80, 0.67, 0.55, and 0.48 after 12, 24, 60, and 120 months, respectively. Histological characteristics at the time of diagnosis predicted the effect of immunosuppression on renal outcome. All patients were dialysis dependent within 24 months if more than one third of glomeruli were totally sclerosed on the initial histological examination. Interstitial fibrosis also correlated significantly with the risk for progression to renal failure. Conversely, long-term dialysis-free survival was significantly more likely in patients with a greater number of crescents than in those with a low number of crescents. In conclusion, plasmapheresis does not add to the improvement in outcome reached by immunosuppression alone. Crescents on initial histological examination correlate with a favorable outcome. However, 90% of patients who initially have glomerular sclerosis present become dialysis dependent. Overall, approximately 50% of patients are alive and off dialysis therapy 10 years after the diagnosis of type 11 or type III RPGN using immunosuppression with cyclophosphamide and prednisone. (C) 2002 by the National Kidney Foundation, Inc.

Item Type: Article
Uncontrolled Keywords: PLASMA-EXCHANGE; NECROTIZING GLOMERULONEPHRITIS; MICROSCOPIC POLYANGIITIS; WEGENERS-GRANULOMATOSIS; RENAL INVOLVEMENT; THERAPY; SURVIVAL; RELAPSE; glomerulonephritis (GN); rapidly progressive glomerulonephritis (RPGN); Wegener's granulomatosis; vasculitis; plasmapheresis; immunosuppression; histology; prospective study; random allocation
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Abteilung für Nephrologie
Depositing User: Dr. Gernot Deinzer
Date Deposited: 15 Nov 2021 10:32
Last Modified: 15 Nov 2021 10:32
URI: https://pred.uni-regensburg.de/id/eprint/40678

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