title: Low-dose rituximab is effective in pemphigus.
abstract: BACKGROUND
  Rituximab, an anti-CD20 antibody, was shown in open series studies to be effective in treating pemphigus at a dose of 4 × 375 mgm(-2) as approved for B-cell malignancies.
  
  
  OBJECTIVE
  We investigated whether a lower dose of rituximab is also effective for pemphigus.
  
  
  METHODS
  Patients with pemphigus were treated with a single course of two infusions of rituximab (500 mg each) at an interval of 2 weeks. Clinical consensus late end points, B-cell number, desmoglein 1 and desmoglein 3 indices were monitored.
  
  
  RESULTS
  We enrolled 15 patients in the study: three with pemphigus foliaceus (PF) and 12 with pemphigus vulgaris (PV). The follow-up was 32-152 weeks (median 94). All 15 patients responded to therapy. Eight patients achieved complete remission in a median period of 5 weeks (four on minimal therapy, four off therapy). Seven patients achieved partial remission in a median period of 34·5 weeks (five on minimal therapy, two off therapy). Relapses (40%) were seen between 53 and 103 weeks (median 97) after start of therapy. B-cell numbers dropped to <1% after first infusion, and remained undetectable in patients with sustained remission. The antidesmoglein 1 index correlated well with the clinical severity in PF, but this was less obvious in PV.
  
  
  CONCLUSIONS
  A low dose of rituximab is an effective and safe treatment for pemphigus. Relapses may occur, mostly at the end of the second year. Cost-effectiveness studies with a long follow-up are required to determine the proper dosage of this expensive drug in pemphigus.
authors.affiliation: Departments of Dermatology Laboratory Medicine, Centre for Blistering Diseases, University Medical Centre Groningen, University of Groningen, PO Box 30001, 9700 RB Groningen, the Netherlands. b.horvath@umcg.nl
authors.forename: B
authors.initials: B
authors.lastname: Horváth
authors.forename: J
authors.initials: J
authors.lastname: Huizinga
authors.forename: H H
authors.initials: HH
authors.lastname: Pas
authors.forename: A B
authors.initials: AB
authors.lastname: Mulder
authors.forename: M F
authors.initials: MF
authors.lastname: Jonkman
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000906:Antibodies / Q000378:metabolism; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage* / Q000009:adverse effects; D018951:Antigens, CD20 / Q000378:metabolism; D001402:B-Lymphocytes / Q000187:drug effects; D003879:Dermatologic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D051183:Desmoglein 1 / Q000276:immunology; D051184:Desmoglein 3 / Q000276:immunology; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D056687:Off-Label Use; D010392:Pemphigus / Q000188:drug therapy*; D011446:Prospective Studies; D012008:Recurrence; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D000906:Antibodies; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D003879:Dermatologic Agents; D051183:Desmoglein 1; D051184:Desmoglein 3; D007155:Immunologic Factors; D000069283:Rituximab