Abstract
Purpose: :
To evaluate the effectiveness and toxicity of current immunosuppressive regimens, including mycophenolate mofetil and combination therapy, in the treatment of ocular mucous membrane pemphigoid.
Methods: :
Retrospective review of the clinical courses of 223 eyes of 115 patients receiving immunosuppression between 1994 –2004. For less active disease, treatment was ‘stepped–up’ using therapies based on dapsone or sulphapyridine before azathioprine or mycophenolate, followed by cyclophosphamide or combination therapy. For rapidly advancing disease a reverse, ‘step–down’ approach was used. Control of inflammation was graded as a success (S), qualified success (QS) or failure (F).
Results: :
In 84% of patients, inflammation was controlled by the end of the study. At least 6 months remission off treatment occurred in 13 patients (11%). Of the 387 treatment episodes, 51% were successful, 27% qualified successes and 22% failures. The most successful therapies were based on cyclophosphamide (69%S, 20%QS, 11%F) (n=74) followed by mycophenolate (56%S, 24%QS, 20%F) (n=46), azathioprine (49%S, 23%QS, 28%F) (n=78), dapsone (48%S, 30%QS, 22%F) (n=115) & sulphapyridine (38%S, 27%QS, 35%F) (n=55). The difference in outcomes between these agents was statistically significant (p=0.01). Combination sulpha–myelosuppressive agent therapy increased the response rate from 73% with single agent therapy, to 86% (p=0.04). 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 visual acuity was ≤6/60 in 17% (37/223) of eyes, pemphigoid being the cause in 13% (29/223). Final best corrected visual acuity was ≤6/60 in 34% (76/223) of eyes, pemphigoid being the cause in 26% (57/223). By the end of the study, cicatrisation had progressed in 41% (92/223) of eyes.
Conclusions: :
Mycophenolate mofetil appears to be an effective and well tolerated immunosuppressant for moderately active ocular mucous membrane pemphigoid. Combination sulpha–myelosuppressive agent therapy is a useful strategy to improve disease control. Cicatrisation and visual acuity can still progress and worsen despite the high proportion of success in controlling inflammation.
Keywords: conjunctivitis • immunomodulation/immunoregulation • clinical (human) or epidemiologic studies: treatment/prevention assessment/controlled clinical trials