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Spectrum and Prognosis of Noninfectious Renal Mixed Cryoglobulinemic GN.
- Source :
-
Journal of the American Society of Nephrology : JASN [J Am Soc Nephrol] 2016 Apr; Vol. 27 (4), pp. 1213-24. Date of Electronic Publication: 2015 Aug 10. - Publication Year :
- 2016
-
Abstract
- Noninfectious mixed cryoglobulinemic GN (MCGN) has been poorly investigated. We analyzed presentation and outcome of 80 patients with biopsy-proven MCGN, which were identified in the retrospective French CryoVas survey. MCGN was related to primary Sjögren's syndrome in 22.5% of patients and to lymphoproliferative disorders in 28.7% of patients, and was defined as essential in 48.8% of patients. At presentation, hematuria, proteinuria ≥1 g/d, hypertension, and renal failure were observed in 97.4%, 84.8%, 85.3%, and 82.3% of cases, respectively. Mean±eGFR was 39.5±20.4 ml/min per 1.73 m(2) Membranoproliferative GN was the predominant histologic pattern, observed in 89.6% of cases. Renal interstitium inflammatory infiltrates were observed in 50% of cases. First-line treatment consisted of steroids alone (27.6%) or in association with rituximab (21.1%), alkylating agents (36.8%) or a combination of cyclophosphamide and rituximab (10.5%). After a mean follow-up of 49.9±45.5 months, 42.7% of patients relapsed with a renal flare in 75% of cases. At last follow-up, mean eGFR was 50.2±26.1 ml/min per 1.73 m(2)with 9% of patients having reached ESRD; 59% and 50% of patients achieved complete clinical and renal remission, respectively. A rituximab+steroids regimen prevented relapses more effectively than steroids alone or a cyclophosphamide+steroids combination did, but was associated with a higher rate of early death when used as first-line therapy. Severe infections and new-onset B-cell lymphoma occurred in 29.1% and 8.9% of cases, respectively; 24% of patients died. In conclusion, noninfectious MCGN has a poor long-term outcome with severe infections as the main cause of death.<br /> (Copyright © 2016 by the American Society of Nephrology.)
- Subjects :
- Cyclophosphamide therapeutic use
Female
Humans
Immunosuppressive Agents therapeutic use
Male
Middle Aged
Prognosis
Retrospective Studies
Rituximab therapeutic use
Cryoglobulinemia complications
Cryoglobulinemia diagnosis
Cryoglobulinemia drug therapy
Glomerulonephritis, Membranoproliferative complications
Glomerulonephritis, Membranoproliferative diagnosis
Glomerulonephritis, Membranoproliferative drug therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1533-3450
- Volume :
- 27
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Journal of the American Society of Nephrology : JASN
- Publication Type :
- Academic Journal
- Accession number :
- 26260165
- Full Text :
- https://doi.org/10.1681/ASN.2015020114