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G. P. Williams, C. F. Radford, V. P. Saw, J. K. G. Dart, S. Rauz; Presentation and Disease Progression in Established Ocular Mucous Membrane Pemphigoid. Invest. Ophthalmol. Vis. Sci. 2008;49(13):803. doi: https://doi.org/.
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To assess disease progression and management strategies amongst patients with established ocular mucous membrane pemphigoid (OMMP) presenting to tertiary referral centres.
A retrospective clinical notes review was undertaken in 24 consecutive patients (48 eyes) aged 39-91 (median 69) years, with a documented history of OMMP of >5 years. All had been referred to two tertiary referral centres for further management. Patients were followed for a total of 24-months. Ethical approval was obtained for this study.
At initial visit, 12/44 eyes (27%) documented had conjunctival inflammation, 11/24 patients (46%) were on existing immunosuppression and 13 (54%) had undergone previous lid surgery for OMMP. Of the eyes where Mondino and Foster staging was documented, staging was gauged as Mondino I 26%, II 9%, III 30% and IV 35% and Foster I 4%, II 7%, III 74% and IV 15%. After excluding ocular co-morbidities that may affect vision, the majority of eyes had visual acuities of >6/18 (n=22/36 (61%)) and <3/60 (n=10 (28%)). Ten patients required increase in immunosuppression at the initial visit, with a further 5 requiring increase during the following 12 months. No patients required further increase between 12 and 24 months. Surgical intervention was performed within the first 12 months in 5 (21%) patients and a further 3 (12.5%) within the next 12 months. Although progression of OMMP was seen during the first 12 months of follow up (Mondino, 5/26 eyes (19%), Foster 9/32 eyes (28%)), progression was less pronounced during 12-24 months follow-up (Mondino, 1/20 (5%), Foster 3/20 (5%)).
The majority of patients that present to tertiary referral centres with established OMMP have advanced disease, often with poor visual acuity, a history of multiple procedures and widespread use of immunosuppression. Despite this, increase in immunosuppression was required in a high proportion of our cohort in order to halt further progression. This subgroup of patients pose a particular challenge and highlight the need for aggressive immunosuppressive intervention at outset, particularly when surgical intervention is indicated.
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