Autoimmune Diseases and Ocular Cicatricial Pemphigoid

Abstract

Abstract: : Background: Ocular Cicatricial Pemphigoid (OCP) is a progressive autoimmune disorder, occurring late in life and characterized by recurrent episodes of inflammation, blistering and progressive conjunctival fibrosis that damages normal eye architecture and can lead to blindness; however the etiology of this disease remains to be elucidated. Many autoimmune diseases share genes that act in tandem as polygenic risk factors for autoimmunity. Purpose: To determine the frequency and distribution of autoimmune diseases in patients with OCP and a comparison group of General Eye Service patients. Methods: Retrospective analysis of the medical records of 68 randomly selected patients of the Massachusetts Eye and Ear Infirmary, with biopsy proven OCP. A similarly aged comparison group (n=99) was randomly chosen from patients attending the General Eye Service. Patient confidentiality was maintained. All prior medical and ocular diagnoses were extracted from available records for both groups of patients. Clearly documented disease entities, established as true autoimmune diseases as defined in Arthritis Rheum, Vol. 41(3).March 1998.400–405, were counted. Results: : 6 of 68 (8.8%) OCP patients had one or more documented autoimmune disease compared to 3 of 99 (3.0%) patients in the comparison group (p=0.16, Fisher exact test). Autoimmune diseases in the OCP patients included: rheumatoid arthritis (RA), MS, Sjogrens, and Hashimoto’s thyroiditis. RA occurred in (4 of 68 patients) 5.9% of OCP patients and 1 patient had >1 autoimmune diseases documented. Autoimmune diseases occurring in the comparison group included sarcoid, primary biliary cirrhosis and scleroderma. 1(1%) patient of the comparison group had >1 autoimmune disease documented. Conclusions: The prevalence of autoimmune disease in the general US population has been reported at 3%, comparable to the comparison group. The possible increased prevalence of autoimmune diseases in the OCP population along with the preponderance of RA in these patients may eventually help establish the genetic determinants in this rare disease and lead to more effective therapies.

Keywords: autoimmune disease • clinical (human) or epidemiologic studies: prevalence/incidence • anterior segment