title: Safety and efficacy of rituximab in nonviral cryoglobulinemia vasculitis: data from the French Autoimmunity and Rituximab registry.
abstract: OBJECTIVE
  Management of nonviral cryoglobulinemia vasculitis has yet to be defined. Rituximab has emerged as a novel and promising therapeutic alternative, but data are scarce. Our objective was to evaluate the safety and efficacy of rituximab in nonviral cryoglobulinemia vasculitis in off-trial real-life patients.
  
  
  METHODS
  Prospective data from the French AutoImmunity and Rituximab (AIR) registry, which includes data on patients with autoimmune disorders treated with rituximab in off-label conditions, were analyzed.
  
  
  RESULTS
  Twenty-three patients received treatment with rituximab for cryoglobulinemia vasculitis. Tolerance was marked by the occurrence of side effects in almost half of the patients, including severe infections in 6 (26%) of 23, with a rate of 14.1 per 100 patient-years. These infections occurred in a particular subset of patients ages>70 years, with essential type II mixed cryoglobulinemia and renal failure with a glomerular filtration rate of <60 ml/minute, and receiving high-dose corticosteroids. Three of these patients died. In contrast, clinical and immunologic efficacy was noted in all evaluable patients. Clinical relapses occurred in half of the patients after a median time of 13.5 months following rituximab administration, and were more frequent in patients refractory to previous immunosuppressive therapy than in previously untreated patients.
  
  
  CONCLUSIONS
  Data from the AIR registry show a dramatic efficacy and a steroid-sparing effect of rituximab, but also show the occurrence of severe infections in elderly patients with renal failure and high-dose steroids. The role of rituximab in nonviral cryoglobulinemia vasculitis remains to be defined in well-designed randomized controlled trials.
authors.affiliation: Groupe Hospitalier Pitié Salpétrière, Assistance Publique Hôpitaux de Paris, Université Pierre et Marie Curie Paris 6, Paris, France.
authors.forename: B
authors.initials: B
authors.lastname: Terrier
authors.forename: D
authors.initials: D
authors.lastname: Launay
authors.forename: G
authors.initials: G
authors.lastname: Kaplanski
authors.forename: A
authors.initials: A
authors.lastname: Hot
authors.forename: C
authors.initials: C
authors.lastname: Larroche
authors.forename: P
authors.initials: P
authors.lastname: Cathébras
authors.forename: B
authors.initials: B
authors.lastname: Combe
authors.forename: J P
authors.initials: JP
authors.lastname: de Jaureguiberry
authors.forename: O
authors.initials: O
authors.lastname: Meyer
authors.forename: T
authors.initials: T
authors.lastname: Schaeverbeke
authors.forename: A
authors.initials: A
authors.lastname: Somogyi
authors.forename: L
authors.initials: L
authors.lastname: Tricot
authors.forename: T
authors.initials: T
authors.lastname: Zénone
authors.forename: P
authors.initials: P
authors.lastname: Ravaud
authors.forename: J E
authors.initials: JE
authors.lastname: Gottenberg
authors.forename: X
authors.initials: X
authors.lastname: Mariette
authors.forename: P
authors.initials: P
authors.lastname: Cacoub
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage / Q000627:therapeutic use*; D018951:Antigens, CD20; D015551:Autoimmunity; D003449:Cryoglobulinemia / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D004305:Dose-Response Relationship, Drug; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D000069283:Rituximab; D016896:Treatment Outcome; D014657:Vasculitis / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D007155:Immunologic Factors; D000069283:Rituximab