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Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey.

Benjamin Terrier 1 Evguenia Krastinova 2 Isabelle Marie 3, 4 David Launay 5 Adeline Lacraz 6 Pauline Belenotti 7 Luc de Saint-Martin 8, 9 Thomas Quemeneur 10 Antoine Huart 11 Fabrice Bonnet 12 Guillaume Le Guenno 13 Jean-Emmanuel Kahn 14 Olivier Hinschberger Patricia Rullier 15 Elisabeth Diot 16 Estibaliz Lazaro 17 Franck Bridoux 18, 19 Thierry Zénone 20 Fabrice Carrat 21 Olivier Hermine 22 Jean-Marc Léger 23 Xavier Mariette 24 Patricia Senet 25 Emmanuelle Plaisier 26 Patrice Cacoub 27, 28 
Abstract : Data on the clinical spectrum and therapeutic management of noninfectious mixed cryoglobulinemia vasculitis (CryoVas) in the era of hepatitis C virus screening are lacking. We analyzed data from 242 patients with noninfectious mixed CryoVas included in the French multicenter CryoVas survey. Baseline manifestations were purpura (75%), peripheral neuropathy (52%), arthralgia or arthritis (44%), glomerulonephritis (35%), cutaneous ulcers (16%), and cutaneous necrosis (14%). A connective tissue disease was diagnosed in 30% and B-cell non-Hodgkin lymphoma in 22%, whereas the CryoVas was considered to be essential in 48%. With the use of Cox-marginal structural models, rituximab plus corticosteroids showed the greater therapeutic efficacy compared with corticosteroids alone and alkylating agents plus corticosteroids to achieve complete clinical, renal, and immunologic responses and a prednisone dosage < 10 mg/d at 6 months. However, this regimen was also associated with severe infections, particularly when high doses of corticosteroids were used, whereas death rates did not differ between the therapeutic regimens. The role of each of these strategies remains to be defined in well-designed randomized controlled trials.
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Submitted on : Thursday, January 16, 2014 - 3:52:32 PM
Last modification on : Saturday, October 8, 2022 - 3:32:18 AM

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Benjamin Terrier, Evguenia Krastinova, Isabelle Marie, David Launay, Adeline Lacraz, et al.. Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey.. Blood, 2012, 119 (25), pp.5996-6004. ⟨10.1182/blood-2011-12-396028⟩. ⟨hal-00932280⟩



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