title: Rituximab therapy in refractory neuropsychiatric lupus: current clinical evidence.
abstract: OBJECTIVE
  To review and summarize published information on the effectiveness and safety of rituximab (RTX) in adult patients with refractory neuropsychiatric systemic lupus erythematosus (NPSLE).
  
  
  METHODS
  We describe a patient with persistently active NPSLE, despite conventional therapy, who responded dramatically to RTX. Current evidence on the therapeutic use of RTX in this complex situation is also analyzed through a systematic review of the English-language literature, based on a PubMed search.
  
  
  RESULTS
  Available data on the use of RTX in refractory NPSLE come from a large number of case reports and some open-label studies. Including our case, 35 patients have been well documented. A complete or partial therapeutic response was achieved in 85% of patients after 1 cycle of treatment. A positive correlation between serological markers of disease activity and clinical outcome has also been demonstrated in some of these patients. Clinical improvement was accompanied by a significant reduction in the daily dose of oral corticosteroids. Relapse after RTX treatment was noted in 45% of cases (median 9.5 months; range, 4-33 months). Infections were observed in 29% of patients.
  
  
  CONCLUSIONS
  Evidence for the effectiveness of RTX as induction therapy in NPSLE is based solely on several case reports and noncontrolled trials. Although it is not yet possible to make definite recommendations, the global analysis of these cases supports the off-label use of RTX in cases of severe refractory NPSLE.
authors.affiliation: Department of Rheumatology, Hospital Universitario de Bellvitge-IDIBELL, Barcelona, Spain. fjnarvaez@bellvitgehospital.cat
authors.forename: Javier
authors.initials: J
authors.lastname: Narváez
authors.forename: Valeria
authors.initials: V
authors.lastname: Ríos-Rodriguez
authors.forename: Diana
authors.initials: D
authors.lastname: de la Fuente
authors.forename: Paula
authors.initials: P
authors.lastname: Estrada
authors.forename: Laura
authors.initials: L
authors.lastname: López-Vives
authors.forename: Carmen
authors.initials: C
authors.lastname: Gómez-Vaquero
authors.forename: Joan Miquel
authors.initials: JM
authors.lastname: Nolla
mesh_terms: D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D020945:Lupus Vasculitis, Central Nervous System / Q000188:drug therapy*; D056687:Off-Label Use; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents; D000069283:Rituximab