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C. F. Radford, G. P. Williams, V. P. Saw, S. Rauz, J. K. G. Dart; Presentation, Management and Disease Progression in Early Ocular Mucous Membrane Pemphigoid. Invest. Ophthalmol. Vis. Sci. 2008;49(13):802.
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To assess presentation, disease progression and management amongst patients with early ocular mucous membrane pemphigoid (OMMP), and to trial a clinical data sheet to be used for a forthcoming national incidence study of OMMP.
The clinical notes of OMMP patients referred to two tertiary external disease clinics between August 2003 and July 2006 were reviewed retrospectively. Analysis of cases with no more than three years of symptoms was undertaken.
There were 26 cases (51 eyes), including 9 (35%) patients without any extra-ocular manifestations. 18/20 (90%) of the cases undergoing conjunctival biopsy had a positive result. One, two and three year follow up was available for 23, 13 and 8 patients respectively. At the initial visit, 22/26 (85%) patients had conjunctival inflammation, 7 (27%) were already on immunosuppressive therapy and 3 (12%) had had lid surgery for OMMP. 40/51 (78%) eyes could be assessed as Mondino stage I (40%), II (35%), III (20%) and IV (5%) and 51 eyes at Foster stage I (17%), II (12%), III (67%) and IV (4%). After excluding eyes with other possible causes of reduced visual acuity (n=18), visual acuities of 6/9 or better were recorded in 27/33 (82%) eyes. 15/26 (58%) patients needed an increase in immunosuppression at the initial visit, with a further 5 (19%) and 2 (8%) having this during the following 12 and 24 months respectively. Surgical intervention was carried out within 12 months of the initial visit (10/26, 39%) or subsequently (3/26, 12%). 22/29 (76%) eyes progressed according to Mondino staging, but this could only be assessed for 29/51 (57%) eyes. 12/51 (24%) eyes progressed according to Foster staging. Final visual acuities for eyes without other causes of visual loss were 6/9 or better in 23/33 (70%). Defining progression as a change in Mondino and/or Foster stage in either eye, and/or loss of at least two lines of visual acuity in either eye due to OMMP, 15/26 (58%) cases progressed.
Fornix depth measurements were not routinely documented in the clinical notes, hindering the use of Mondino staging. A combined Mondino and Foster definition that includes loss of acuity attributable to OMMP may be more informative when defining disease progression in retrospective reviews. A high proportion of patients needed an increase in immunosuppression and/or surgery following referral, highlighting the need for prompt referral of early cases of OMMP to tertiary clinics.
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