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Spectrum and Prognosis of Noninfectious Renal Mixed Cryoglobulinemic GN

Mohamad Zaidan 1 Benjamin Terrier 2, 3 Agnieszka Pozdzik 4, 5 Thierry Frouget 6 Nathalie Rioux-Leclercq 7 Christian Combe 8, 9, 10 Sébastien Lepreux 11 Aurélie Hummel 1 Laure-Hélène Nöel 12, 13 Isabelle Marie 14 Bruno Legallicier Arnaud François 15 Antoine Huart 16 David Launay 17, 18, 19 Gilles Kaplanski Franck Bridoux 20 Philippe Vanhille 21 Raifah Makdassi Jean-François Augusto 22 Philippe Rouvier Alexandre Karras 23 Chantal Jouanneau 24 Marie-Christine Verpont Patrice Callard 25 Fabrice Carrat 26, 27 Olivier Hermine 28, 29, 30 Jean-Marc Léger 31 Xavier Mariette 32 Patricia Senet 33 David Saadoun 34, 35 Pierre Ronco 36, 37, 24 Isabelle Brochériou 24, 25 Patrice Cacoub 34, 35 Emmanuelle Plaisier 37, 24
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 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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Submitted on : Wednesday, April 27, 2016 - 10:15:26 AM
Last modification on : Friday, October 16, 2020 - 3:21:31 AM

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Mohamad Zaidan, Benjamin Terrier, Agnieszka Pozdzik, Thierry Frouget, Nathalie Rioux-Leclercq, et al.. Spectrum and Prognosis of Noninfectious Renal Mixed Cryoglobulinemic GN. Journal of the American Society of Nephrology, American Society of Nephrology, 2016, 27 (4), pp.1213--1224. ⟨10.1681/ASN.2015020114⟩. ⟨hal-01308003⟩

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