Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey. - Université de Bretagne Occidentale Access content directly
Journal Articles Blood Year : 2012

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

David Launay
Olivier Hinschberger
  • Function : Author
Patricia Senet
  • Function : Author

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.

Dates and versions

hal-00932280 , version 1 (16-01-2014)

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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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