title: Tolerance and efficacy of rituximab and changes in serum B cell biomarkers in patients with systemic complications of primary Sjögren's syndrome.
abstract: OBJECTIVE
  To investigate the safety and efficacy of rituximab (RTX) for systemic symptoms in patients with primary Sjögren's syndrome (pSS), and changes in B cell biomarkers.
  
  
  METHODS
  The records of 16 patients with pSS according to the American European consensus group criteria were reviewed retrospectively.
  
  
  RESULTS
  Patients, all women, had a median age of 58.5 (range 41-71) years and a disease duration of 9.5 (range 0-25) years. RTX was prescribed for lymphoma (n = 5), refractory pulmonary disease with polysynovitis (n = 2), severe polysynovitis (n = 2), mixed cryoglobulinaemia (n = 5), thrombocytopenia (n = 1) and mononeuritis multiplex (n = 1). The median follow-up duration was 14.5 (range 2-48) months. Three patients experienced adverse events, including one mild serum sickness-like reaction with the presence of human antichimeric antibodies. Efficacy of treatment was observed in 4 of 5 patients with lymphomas and in 9 of 11 patients with systemic involvement. Dryness was improved in only a minority of patients. Corticosteroid dose was reduced in 11 patients. RTX induced decreased rheumatoid factor, gamma-globulin and beta2-microglobulin levels, and the level of B cell activating factor of the tumour necrosis factor family (BAFF) increased concomitantly with B cell depletion. Five patients were re-treated, with good efficacy and tolerance, except for one with probable serum sickness-like reaction.
  
  
  CONCLUSIONS
  This study shows good efficacy and fair tolerance of RTX for systemic features. In addition, RTX allows for a marked reduction in corticosteroid use. Except for BAFF, the level of which increases, serum B cell biomarker levels decrease after taking RTX. Controlled trials should be performed to confirm the efficacy of RTX in pSS.
authors.affiliation: Department of Rheumatology, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université paris-Sud 11, INSERM U802, Le Kremlin Bicêtre, France.
authors.forename: Raphaèle
authors.initials: R
authors.lastname: Seror
authors.forename: Christelle
authors.initials: C
authors.lastname: Sordet
authors.forename: Loic
authors.initials: L
authors.lastname: Guillevin
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Charles
authors.initials: C
authors.lastname: Masson
authors.forename: Marc
authors.initials: M
authors.lastname: Ittah
authors.forename: Sophie
authors.initials: S
authors.lastname: Candon
authors.forename: Véronique
authors.initials: V
authors.lastname: Le Guern
authors.forename: Achille
authors.initials: A
authors.lastname: Aouba
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; D000911:Antibodies, Monoclonal / Q000009:adverse effects / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use; D018501:Antirheumatic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D001402:B-Lymphocytes / Q000187:drug effects* / Q000276:immunology; D015415:Biomarkers / Q000097:blood; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D016393:Lymphoma, B-Cell / Q000188:drug therapy; D008875:Middle Aged; D012189:Retrospective Studies; D000069283:Rituximab; D012859:Sjogren's Syndrome / Q000188:drug therapy* / Q000276:immunology; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D018501:Antirheumatic Agents; D015415:Biomarkers; D005938:Glucocorticoids; D000069283:Rituximab