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10.1681/ASN.2015020114

http://scihub22266oqcxt.onion/10.1681/ASN.2015020114
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C4814186!4814186!26260165
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suck abstract from ncbi


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pmid26260165      J+Am+Soc+Nephrol 2016 ; 27 (4): 1213-24
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  • Spectrum and Prognosis of Noninfectious Renal Mixed Cryoglobulinemic GN #MMPMID26260165
  • Zaidan M; Terrier B; Pozdzik A; Frouget T; Rioux-Leclercq N; Combe C; Lepreux S; Hummel A; Noël LH; Marie I; Legallicier B; François A; Huart A; Launay D; Kaplanski G; Bridoux F; Vanhille P; Makdassi R; Augusto JF; Rouvier P; Karras A; Jouanneau C; Verpont MC; Callard P; Carrat F; Hermine O; Léger JM; Mariette X; Senet P; Saadoun D; Ronco P; Brochériou I; Cacoub P; Plaisier E
  • J Am Soc Nephrol 2016[Apr]; 27 (4): 1213-24 PMID26260165show ga
  • 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?m2. 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?m2 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.
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