Immunosuppressive Therapy for Ocular Mucous Membrane Pemphigoid: Strategies and Outcomes
Presented in part as a poster at: Association for Research in Vision and Ophthalmology Annual Meeting, May 2006, Fort Lauderdale, Florida. Presented at: Royal Australian and New Zealand College of Ophthalmologists Annual Meeting, November 2005, Hobart, Australia.
Received 3 June 2006; received in revised form 22 April 2007; accepted 23 April 2007. published online 28 July 2007.
Purpose
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.
Design
Retrospective, noncomparative, interventional case series.
Participants
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.
Main Outcome Measures
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.
Available online: July 26, 2007.
1Cornea and External Disease Service, Moorfields Eye Hospital, London, United Kingdom.
2Institute of Ophthalmology, University College London, London, United Kingdom.
3Statistics Department, Research and Development, Moorfields Eye Hospital, London, United Kingdom.
Correspondence to Valerie P. J. Saw, FRANZCO, Cornea and External Disease Service, Moorfields Eye Hospital, 162 City Road, London EC1V 2PD, United Kingdom.
Manuscript no. 2006-604.
The authors have no proprietary interest in any of the products described in the article.
Supported by the Special Trustees of Moorfields Eye Hospital; a Royal Australian and New Zealand College of Ophthalmologists (Sydney, Australia) Advanced Medical Optics Scholarship; and a University College London Graduate School Research Scholarship (VPJS).
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