title: Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey.
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.
authors.affiliation: Department of Internal Medicine, Groupe Hospitalier Pitié-Salpetrière, Assistance Publique des Hôpitaux de Paris, Université Pierre et Marie Curie, 47-83 Boulevard de l’Hôpital, Paris, France.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.forename: Evguenia
authors.initials: E
authors.lastname: Krastinova
authors.forename: Isabelle
authors.initials: I
authors.lastname: Marie
authors.forename: David
authors.initials: D
authors.lastname: Launay
authors.forename: Adeline
authors.initials: A
authors.lastname: Lacraz
authors.forename: Pauline
authors.initials: P
authors.lastname: Belenotti
authors.forename: Luc
authors.initials: L
authors.lastname: de Saint-Martin
authors.forename: Thomas
authors.initials: T
authors.lastname: Quemeneur
authors.forename: Antoine
authors.initials: A
authors.lastname: Huart
authors.forename: Fabrice
authors.initials: F
authors.lastname: Bonnet
authors.forename: Guillaume
authors.initials: G
authors.lastname: Le Guenno
authors.forename: Jean-Emmanuel
authors.initials: JE
authors.lastname: Kahn
authors.forename: Olivier
authors.initials: O
authors.lastname: Hinschberger
authors.forename: Patricia
authors.initials: P
authors.lastname: Rullier
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Diot
authors.forename: Estibaliz
authors.initials: E
authors.lastname: Lazaro
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
authors.forename: Thierry
authors.initials: T
authors.lastname: Zénone
authors.forename: Fabrice
authors.initials: F
authors.lastname: Carrat
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Léger
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
authors.forename: Patricia
authors.initials: P
authors.lastname: Senet
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Plaisier
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000009:adverse effects; D000368:Aged; D000465:Algorithms; D000911:Antibodies, Monoclonal / Q000008:administration & dosage / Q000009:adverse effects; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage / Q000009:adverse effects; D015331:Cohort Studies; D003131:Combined Modality Therapy; D003449:Cryoglobulinemia / Q000150:complications* / Q000453:epidemiology / Q000628:therapy*; D003625:Data Collection; D005260:Female; D006801:Humans; D007239:Infections / Q000150:complications / Q000453:epidemiology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D000069283:Rituximab; D016896:Treatment Outcome; D014657:Vasculitis / Q000150:complications* / Q000453:epidemiology / Q000628:therapy*
chemical_list: D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab