title: Therapy with rituximab for autoimmune pemphigus: results from a single-center observational study on 42 cases with long-term follow-up.
abstract: BACKGROUND
  Rituximab induces depletion of B cells and has shown efficacy in antibody-mediated autoimmune disorders. In studies on small series of patients with pemphigus, rituximab administration results in significant improvement. However, differences in inclusion criteria, treatment protocols, and follow-up make it difficult to derive uniform conclusions.
  
  
  OBJECTIVE
  We sought to test the efficacy and tolerability of rituximab as adjuvant therapy to corticosteroids in the treatment of pemphigus.
  
  
  METHODS
  In all, 42 patients with pemphigus were treated with rituximab and followed up for up to 5 years. No additional immunosuppressive agents were used. Steroids were rapidly tapered. Outcomes were the proportion of patients who achieved a complete response on or off therapy, the rate of discontinuation of corticosteroid within 6 months, length of remission, time to relapses, and occurrence of adverse events.
  
  
  RESULTS
  In all, 36 of 42 patients (86%; 95% confidence interval 75%-96%) achieved a complete response on or off therapy and discontinued steroids within 6 months from induction therapy. Six patients had a complete response off therapy with an additional infusion of rituximab 6 months after initial treatment. Twenty patients experienced a total of 34 relapses; the time to relapse was 8 to 64 months. Every relapse was treated with rituximab (500 mg) without corticosteroids, which induced a new complete response. No serious adverse events were observed.
  
  
  CONCLUSIONS
  Lack of a control group is a limitation.
  
  
  CONCLUSIONS
  Rituximab therapy induces prolonged clinical remission in patients with pemphigus. Coadministration of other immunosuppressive agents is not necessary. Relapses can be managed with additional infusions administered on demand.
authors.affiliation: Department of Immunodermatology, Istituto Dermopatico dell'Immacolata, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy. g.cianchini@idi.it
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Cianchini
authors.forename: Francesca
authors.initials: F
authors.lastname: Lupi
authors.forename: Cinzia
authors.initials: C
authors.lastname: Masini
authors.forename: Rosamaria
authors.initials: R
authors.lastname: Corona
authors.forename: Pietro
authors.initials: P
authors.lastname: Puddu
authors.forename: Ornella
authors.initials: O
authors.lastname: De Pità
mesh_terms: D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage* / Q000009:adverse effects; D004359:Drug Therapy, Combination / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy* / Q000276:immunology; D011241:Prednisone / Q000008:administration & dosage*; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D005938:Glucocorticoids; D007155:Immunologic Factors; D000069283:Rituximab; D011241:Prednisone