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C. F. Radford, V. P. Saw, S. Rauz, J. K. G. Dart; Ocular Mucous Membrane Pemphigoid: Delayed Diagnosis. Invest. Ophthalmol. Vis. Sci. 2009;50(13):1527.
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Ocular Mucous Membrane Pemphigoid (ocMMP) is a rare, sight-threatening immune-mediated disease for which early diagnosis and appropriate treatment are essential. ocMMP is often associated with extra-ocular manifestations, with oral involvement being the most common.A series of newly diagnosed cases were reviewed to evaluate clinical signs, disease stage, treatment and duration of symptoms at the time of diagnosis.
Cases of ocMMP were reported via the British Ophthalmological Surveillance Unit, over a 10 month period. Reporting ophthalmologists completed a clinical data sheet detailing medical history, disease onset, extra-ocular manifestations, visual acuities, treatments, biopsy results, degree of cicatrisation and inflammation and corneal changes.
Data was received for 33 cases from 16 hospitals in the UK. Patients were aged between 24 and 90 years, with an average age of 68 years, and 61% were male. 17/33 (52%) cases were purely ocular. The duration of symptoms ranged from 1 week to 10 years, with an average and median of 707 and 303 days respectively. Absence of preceding extraocular manifestations was significantly associated with diagnostic delay of more than one year (p=0.034). At the time of diagnosis 11/60 (18%) eyes had a visual acuity of 0.5 or less without any other attributable cause, 22/63 (35%) eyes had moderate or severe conjunctival inflammation and 19/61 (31%) and 40/65 (62%) could be classified as stages 3 or 4 according to Mondino or Foster classification respectively. Only 1/33 patients was on immunosuppressive therapy; for 18/33 (54%) immunosuppressive treatment was initiated or stepped up at the time of diagnosis.
Long delays in diagnosis of ocMMP are common, particularly in the absence of extraocular manifestations. At the time of diagnosis many patients already have moderate or severe disease. These findings highlight the importance of increasing awareness of this disease and prompt referral to centres with expertise in immunosuppression.
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