title: Combined treatment with immunoadsorption and rituximab leads to fast and prolonged clinical remission in difficult-to-treat pemphigus vulgaris.
abstract: BACKGROUND
  Pemphigus vulgaris (PV) is a potentially life-threatening autoimmune bullous disorder which is characterized by blisters and erosions of the skin and mucous membranes. A frequently applied first-line therapy for PV consists of systemic corticosteroids (CS) combined with immunosuppressive agents. In refractory cases, novel therapeutic strategies such as immunoadsorption (IA) and the anti-CD20 antibody rituximab (Rtx) aim at directly interfering with pathogenic autoantibodies (auto-Abs).
  
  
  OBJECTIVE
  To investigate the long-term efficacy of IA in combination with Rtx in patients with difficult-to-treat PV, we assessed the clinical response to treatment by monitoring the Autoimmune Bullous Skin Disorder Intensity Score, IgG auto-Abs against desmoglein 1 and 3 (Dsg1 and Dsg3) and the dose of systemic CS.
  
  
  METHODS
  We retrospectively analysed clinical and serological parameters of 10 patients with difficult-to-treat PV who received IA at 4-week intervals, followed by Rtx either twice at 1000 mg or four times at 375mg m(-2) . During a 12-month follow-up period, CS were tapered according to the individual clinical status.
  
  
  RESULTS
  Six months after the first IA treatment eight of 10 patients were in complete remission on therapy while one patient showed a partial response and one patient was unresponsive to the treatment. At 12 months, six of eight patients were in complete remission on therapy, one patient showed stable disease and one patient had relapsed. Overall, anti-Dsg3 IgG and anti-Dsg1 IgG auto-Abs correlated well with the clinical activity and systemic CS were tapered gradually.
  
  
  CONCLUSIONS
  The present findings show that the combination of IA and Rtx induces rapid clinical remission and long-term control in difficult-to-treat pemphigus.
authors.affiliation: Department of Dermatology and Allergology, Philipps-Universität, D-35043 Marburg, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Behzad
authors.forename: C
authors.initials: C
authors.lastname: Möbs
authors.forename: A
authors.initials: A
authors.lastname: Kneisel
authors.forename: M
authors.initials: M
authors.lastname: Möller
authors.forename: J
authors.initials: J
authors.lastname: Hoyer
authors.forename: M
authors.initials: M
authors.lastname: Hertl
authors.forename: R
authors.initials: R
authors.lastname: Eming
mesh_terms: D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001323:Autoantibodies / Q000378:metabolism; D002908:Chronic Disease; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D007155:Immunologic Factors / Q000627:therapeutic use*; D007163:Immunosorbent Techniques; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000097:blood / Q000628:therapy*; D011239:Prednisolone; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007074:Immunoglobulin G; D007155:Immunologic Factors; D000069283:Rituximab; D011239:Prednisolone