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S. Rauz, P.G. Maddison, J.K. Dart; Evaluation of the Progression of Ocular Cicatricial Pemphigoid in Young Patients . Invest. Ophthalmol. Vis. Sci. 2003;44(13):3768.
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Purpose : Ocular cicatricial pemphigoid (OCP) is a sight-threatening autoimmune disease that may lead to severe conjunctival cicatrisation and keratopathy. The peak age of onset is in the eighth decade, although the disease may also occur in younger patients (<60 years). This study was designed to determine the clinical outcome of young patients with OCP and the influence of immunosuppression on the progression when compared with patients in the above 70 age group. Methods: Eighteen patients under the age of 60 years (young), and 18 patients above the age of 70 years (old), with documented disease progression were identified from the External Disease Immunosuppression Database. Ocular stage (Foster, Mondino), visual acuity, and ocular complications (lid, conjunctival, corneal) were evaluated at presentation, at the time when immunosuppression was commenced, and at final follow-up. Disease progression, control of ocular inflammation with systemic immunosuppression, the incidence of extraocular involvement (mucocutaneous lesions) and surgical intervention were also assessed. Results: Patients in the two groups (young and old) were followed-up for 66.7 ± 51.6 and 67.7 ± 48.3 months respectively (mean ± SD). The mean age at the start of immunosuppression was 48.7 ± 8.3 and 78.0 ± 4.4 years. Extraocular involvement was more common in the young than in the older age group (13 (72%), 7 (39%), p<0.05). Ocular staging (Mondino, Foster) at presentation, at the start of immunosuppression, and at final follow up was more advanced in the younger patients than the older patients. There was no statistical difference in visual acuity, individual ocular complications, or incidence of surgical intervention between the two groups throughout the course of the study. Conclusions: Younger patients with OCP present with more severe ocular and systemic disease, and despite immunosuppression, progress more rapidly.
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