title: Rituximab in central nervous system manifestations of patients with primary Sjögren's syndrome: results from the AIR registry.
abstract: OBJECTIVE
  To evaluate the efficacy of rituximab in central nervous system (CNS) manifestations of patients with primary Sjögren's syndrome (pSS).
  
  
  METHODS
  Prospective data from patients with pSS and CNS involvement included in the French AutoImmunity and Rituximab registry were analysed. All patients had diffuse white matter T2-weigted hypersignals. Neurological response was defined as improvement or disappearance of neurological signs.
  
  
  RESULTS
  Eleven patients (mean age 55 years [38-77]) were treated with rituximab for their neurological involvement. The mean duration of pSS was 9 years (4-24). Mean baseline ESSDAI score was 17 (5-25). Neurological features were progressive multiple sclerosis-like manifestations (n=6), transverse myelitis (n=1), anxiety and depression disorder (n=1) and cognitive dysfunction (n=3). Mean Expanded Disability Status Score (EDSS) before rituximab was 4 (3-5.5). The mean follow-up was of 13 months (6-58). No neurological change occurred in all 6 patients with multiple sclerosis-like symptoms, in 2/3 patients with cognitive dysfunction or in the patient with anxiety-depression. One patient with depression and cognitive dysfunction disclosed subjective improvement. One patient with transverse myelitis, refractory to cyclophosphamide had an improvement of his walk perimeter (160 meters vs. 116). Mean EDSS score and ESSDAI remained stable.
  
  
  CONCLUSIONS
  Rituximab does not seem to be effective in progressive multiple sclerosis-like manifestations of patients with pSS-related CNS involvement.
authors.affiliation: Department of Internal Medicine, Jean Verdier Hospital, Bondy, France. arsene150@yahoo.fr
authors.forename: Arsene
authors.initials: A
authors.lastname: Mekinian
authors.forename: Philippe
authors.initials: P
authors.lastname: Ravaud
authors.forename: Claire
authors.initials: C
authors.lastname: Larroche
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Bruno
authors.initials: B
authors.lastname: Gombert
authors.forename: Claire
authors.initials: C
authors.lastname: Blanchard-Delaunay
authors.forename: Alain
authors.initials: A
authors.lastname: Cantagrel
authors.forename: Olivier
authors.initials: O
authors.lastname: Fain
authors.forename: Jean
authors.initials: J
authors.lastname: Sibilia
authors.forename: Jacques-Eric
authors.initials: JE
authors.lastname: Gottenberg
authors.forename: Xavier
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*; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D004185:Disability Evaluation; D005260:Female; D005602:France; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D000069283:Rituximab; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents; D000069283:Rituximab