title: Treatment of severe autoimmune blistering skin diseases with combination of protein A immunoadsorption and rituximab: a protocol without initial high dose or pulse steroid medication.
abstract: BACKGROUND
  Skin blistering diseases due to autoantibodies are typically treated with high dose systemic corticosteroids and other conventional immunosuppressants. However, in severe cases, this treatment may not be sufficient to achieve disease control or contraindicated because of comorbidity.
  
  
  METHODS
  We describe 15 patients (pts.) with such diseases: 6 pts. with pemphigus vulgaris, 3 pts. with bullous pemphigoid, 3 pts. with mucous membrane pemphigoid (MMP), one being anti-laminin-332-MMP (AL332-MMP), 2 pts. with pemphigus foliaceus and 1 pt. with epidermolysis bullosa acquisita (EBA). Patients were treated with a combination of protein A immunoadsorption (PAIA, 3-21 treatments) and rituximab (3-6 treatments) in addition to low dose conventional immunosuppression.
  
  
  RESULTS
  All patients showed rapid clinical improvement starting within the first 4 weeks and decline of circulating autoantibody levels. Complete/partial remission was 88%/12% in pemphigus and 71%/29% in subepidermal blistering diseases. Overall relapse rate was 13% with an average follow-up of 22 months. In the AL332-MMP pt. the PAIA/rituximab treatment was stopped because of an oesophagus cancer considered as the paraneoplastic cause of the skin disease.
  
  
  CONCLUSIONS
  Combined treatment with PAIA and rituximab showed rapid and long-lasting response, thereby allowing substantial reduction of dosage of concomitant immunosuppressive medication. We hereby confirm data from other investigators that PAIA/rituximab treatment is a promising therapeutical modality for pemphigus, pemphigoids and EBA, characterized by a favourable ratio of beneficial efficacy and minimized long-term adverse effects.
authors: Clinic for Dermatology and Venereology, Otto-von-Guericke-University Magdeburg, Magdeburg, Germany.
authors: M
authors: M
authors: Kolesnik
authors: E
authors: E
authors: Becker
authors: D
authors: D
authors: Reinhold
authors: A
authors: A
authors: Ambach
authors: M U
authors: MU
authors: Heim
authors: H
authors: H
authors: Gollnick
authors: B
authors: B
authors: Bonnekoh
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D001327:Autoimmune Diseases / Q000628:therapy*; D002985:Clinical Protocols; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage*; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D020551:Pulse Therapy, Drug; D012189:Retrospective Studies; D000069283:Rituximab; D012720:Severity of Illness Index; D012872:Skin Diseases, Vesiculobullous / Q000276:immunology* / Q000628:therapy*; D016060:Sorption Detoxification; D013205:Staphylococcal Protein A
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D013205:Staphylococcal Protein A; D000069283:Rituximab