title: Efficacy of rituximab in primary Sjogren's syndrome with peripheral nervous system involvement: results from the AIR registry.
abstract: OBJECTIVE
  To evaluate rituximab (RTX) in primary Sjögren's syndrome (pSS) with peripheral nervous system (PNS) involvement.
  
  
  METHODS
  Patients with pSS and PNS involvement who were included in the French AIR registry were analysed.
  
  
  RESULTS
  17 patients (age 60 years (44-78 years); 14 were female) were analysed. Neurological improvement was noted in 11 patients (65%) at 3 months. Rankin scale decreased from 3 (1-5) to 2 (1-5), 2 (1-5) and 2 (1-6) after 3, 6 and 9 months (p=0.02). European Sjögren's Syndrome Disease Activity Index decreased from 18 (10-44) to 11 (5-20), 11 (5-29) and 12 (5-30) after 3, 6 and 9 months (p<0.05). RTX was effective in neurological involvement in 9/10 patients with vasculitis or cryoglobulinaemia (90%) (group 1) at 3 months and in 2/7 cases (29%) without cryoglobulinaemia and vasculitis (p=0.03). Rankin and European Sjögren's Syndrome Disease Activity Index scales decreased significantly in group 1.
  
  
  CONCLUSIONS
  RTX seems effective in cryoglobulinaemia or vasculitis-related PNS involvement in pSS.
authors.affiliation: Department of Internal Medicine, Hôpital Jean Verdier, Assistance Publique-Hôpitaux de Paris, Université Paris-Sud 13, Bondy, France.
authors.forename: A
authors.initials: A
authors.lastname: Mekinian
authors.forename: P
authors.initials: P
authors.lastname: Ravaud
authors.forename: P Y
authors.initials: PY
authors.lastname: Hatron
authors.forename: C
authors.initials: C
authors.lastname: Larroche
authors.forename: J
authors.initials: J
authors.lastname: Leone
authors.forename: B
authors.initials: B
authors.lastname: Gombert
authors.forename: M
authors.initials: M
authors.lastname: Hamidou
authors.forename: A
authors.initials: A
authors.lastname: Cantagrel
authors.forename: C
authors.initials: C
authors.lastname: Marcelli
authors.forename: S
authors.initials: S
authors.lastname: Rist
authors.forename: M
authors.initials: M
authors.lastname: Breban
authors.forename: D
authors.initials: D
authors.lastname: Launay
authors.forename: O
authors.initials: O
authors.lastname: Fain
authors.forename: J E
authors.initials: JE
authors.lastname: Gottenberg
authors.forename: X
authors.initials: X
authors.lastname: Mariette
mesh_terms: D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000009:adverse effects / Q000627:therapeutic use*; D018501:Antirheumatic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D003449:Cryoglobulinemia / Q000150:complications / Q000188:drug therapy; D004341:Drug Evaluation / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy* / Q000209:etiology; D012042:Registries; D000069283:Rituximab; D012859:Sjogren's Syndrome / Q000150:complications / Q000188:drug therapy*; D016896:Treatment Outcome; D014657:Vasculitis / Q000150:complications / Q000188:drug therapy
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents; D000069283:Rituximab