title: Immunosuppressive therapy for ocular mucous membrane pemphigoid strategies and outcomes.
abstract: OBJECTIVE
  To evaluate the effectiveness and toxicity of a stepladder immunosuppression strategy, including the use of mycophenolate mofetil and combination therapy, in the treatment of ocular mucous membrane pemphigoid.
  
  
  METHODS
  Retrospective, noncomparative, interventional case series.
  
  
  METHODS
  Two hundred twenty-three eyes of 115 patients.
  
  
  METHODS
  Patients with a diagnosis of ocular mucous membrane pemphigoid commencing immunosuppression between January 1994 and July 2005 were identified. A treatment episode was defined by the use of a particular therapy or combination of therapies.
  
  
  METHODS
  For each treatment episode, success of immunosuppressive therapy in controlling ocular inflammation was graded as a success (S), qualified success (QS), or failure (F). Initial and final visual acuities (VAs), stage of cicatrization (Foster, Mondino), grade of conjunctival inflammation, and side effects were recorded.
  
  
  RESULTS
  In 70% (80/115) of patients, inflammation was controlled by the end of the study. At least 6 months remission off treatment occurred in 16 patients (14%). Of the 388 treatment episodes, 50% were classified as S; 27%, QS; and 23%, F. The most successful therapies were based on cyclophosphamide (S, 69%; QS, 21%; F, 10%), followed by mycophenolate (S, 59%; QS, 22%; F, 19%), azathioprine (S, 47%; QS, 24%; F, 29%), dapsone (S, 47%; QS, 30%; F, 23%), and sulfapyridine (S, 38%; QS, 27%; F, 35%). Combination sulfa-steroid-myelosuppressive agent therapy increased the response from 73% with single-agent therapy to 87%. Side effects were the reason for 29% of changes in therapy. These were most prominent with azathioprine (40%) and least with mycophenolate (15%). Initial best-corrected VA (BCVA) was 6/60 or less in 17% (37/223) of eyes, pemphigoid being the cause in 13% (29/223). Final BCVA was 6/60 or less in 34% (76/223) of eyes, pemphigoid being the cause in 26% (57/223). By the end of the study, Mondino stage cicatrization had progressed in 41% (92/223) of eyes and 53% (61/115) of patients.
  
  
  CONCLUSIONS
  Mycophenolate mofetil seems to be an effective and well-tolerated immunosuppressant for moderately active ocular mucous membrane pemphigoid. Combination sulfa-steroid-myelosuppressive agent therapy in a stepladder regimen is a useful strategy to improve disease control. Cicatrization and VA may still progress and worsen despite adequate control of inflammation.
authors.affiliation: Cornea and External Disease Service, Moorfields Eye Hospital, London, United Kingdom. v.saw@ucl.ac.uk
authors.forename: Valerie P J
authors.initials: VP
authors.lastname: Saw
authors.forename: John K G
authors.initials: JK
authors.lastname: Dart
authors.forename: Saaeha
authors.initials: S
authors.lastname: Rauz
authors.forename: Andrew
authors.initials: A
authors.lastname: Ramsay
authors.forename: Catey
authors.initials: C
authors.lastname: Bunce
authors.forename: Wen
authors.initials: W
authors.lastname: Xing
authors.forename: Patricia G
authors.initials: PG
authors.lastname: Maddison
authors.forename: Melanie
authors.initials: M
authors.lastname: Phillips
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001379:Azathioprine / Q000009:adverse effects / Q000627:therapeutic use; D003229:Conjunctival Diseases / Q000175:diagnosis / Q000188:drug therapy*; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use; D003622:Dapsone / Q000009:adverse effects / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000009:adverse effects / Q000031:analogs & derivatives / Q000627:therapeutic use; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis / Q000188:drug therapy*; D012189:Retrospective Studies; D013427:Sulfapyridine / Q000009:adverse effects / Q000627:therapeutic use; D016896:Treatment Outcome; D014792:Visual Acuity
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D003622:Dapsone; D009173:Mycophenolic Acid; D001379:Azathioprine; D013427:Sulfapyridine