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Relapse rate, renal survival, and cancer morbidity in patients with Wegener's granulomatosis or microscopic polyangiitis with renal involvement.

K W Westman, P G Bygren, H Olsson, J Ranstam and J Wieslander
JASN May 1998, 9 (5) 842-852; DOI: https://doi.org/10.1681/ASN.V95842
K W Westman
Department of Nephrology, University Hospital, Lund, Sweden.
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P G Bygren
Department of Nephrology, University Hospital, Lund, Sweden.
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H Olsson
Department of Nephrology, University Hospital, Lund, Sweden.
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J Ranstam
Department of Nephrology, University Hospital, Lund, Sweden.
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J Wieslander
Department of Nephrology, University Hospital, Lund, Sweden.
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Abstract

Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are both frequently associated with antineutrophil cytoplasmic autoantibodies (ANCA). Immunosuppressive treatment has dramatically improved outcome for these patients, but today we have to deal with the problems of relapses, cases refractory to treatment, and long-term side effects of therapy. This study comprises a consecutive series of 123 patients with WG (n=56) or MPA (n=67) with biopsy-confirmed renal involvement, followed up for a median of 55 mo (range, 0.1 to 273.2 mo). ANCA was detected by enzyme-linked immunosorbent assay in 97% of patients. Nearly half of the patients (46%) relapsed. There was no statistically significant difference in overall relapse rate according to type of ANCA. Renal survival was 78% in patients alive at the end of follow-up. Three variables seemed important for renal survival: serum creatinine, the titer of proteinase 3-ANCA measured by capture enzyme-linked immunosorbent assay, and B thrombocyte count, at time of referral. Cancer incidence data were obtained from the population-based South Swedish Regional Tumor Registry. Standardized morbidity ratio was calculated using expected values from the health care region. We found an 11-fold increase in risk for bladder cancer in patients treated with cyclophosphamide for at least 12 mo. Skin carcinoma had the strongest relationship with azathioprine use for at least 12 mo and with corticosteroid therapy for at least 48 mo. In addition, four patients developed myelodysplastic syndrome and five had carcinoma in situ of the skin. Because the therapeutic regimen used today is not efficient enough to prevent relapses and is associated with a host of side effects, of which the risk for cancer is by far the most important, improved therapy and medical care are needed for patients with WG and MPA.

  • Copyright © 1998 by American Society of Nephrology
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Journal of the American Society of Nephrology
Vol. 9, Issue 5
1 May 1998
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Relapse rate, renal survival, and cancer morbidity in patients with Wegener's granulomatosis or microscopic polyangiitis with renal involvement.
K W Westman, P G Bygren, H Olsson, J Ranstam, J Wieslander
JASN May 1998, 9 (5) 842-852; DOI: 10.1681/ASN.V95842

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Relapse rate, renal survival, and cancer morbidity in patients with Wegener's granulomatosis or microscopic polyangiitis with renal involvement.
K W Westman, P G Bygren, H Olsson, J Ranstam, J Wieslander
JASN May 1998, 9 (5) 842-852; DOI: 10.1681/ASN.V95842
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  • Incidence of ANCA-Associated Primary Renal Vasculitis in the Miyazaki Prefecture: The First Population-Based, Retrospective, Epidemiologic Survey in Japan
  • High Proteinase 3-Anti-Neutrophil Cytoplasmic Antibody (ANCA) Level Measured by the Capture Enzyme-Linked Immunosorbent Assay Method Is Associated with Decreased Patient Survival in ANCA-Associated Vasculitis with Renal Involvement
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  • Glomerulonephritis Recurrence in the Renal Graft
  • Mycophenolate Mofetil for Maintenance Therapy of Wegener's Granulomatosis and Microscopic Polyangiitis: A Pilot Study in 11 Patients with Renal Involvement
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