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L. L. Lim, J. D. Scarborough, J. M. Schwartz, J. R. Smith; Autoimmune Hepatitis and Uveitis: A Possible Association. Invest. Ophthalmol. Vis. Sci. 2008;49(13):3237.
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Autoimmune hepatitis (AIH) is a chronic progressive hepatitis of unknown cause that has been associated with other autoimmune diseases such as thyroiditis and rheumatoid arthritis. Uveitis is also a rare autoimmune condition that may be associated with systemic autoimmune disease. The type of uveitis often fits a specific pattern that is typical for that disease. Here we report 9 cases of AIH and uveitis, raising the possibility that uveitis may be an extra-hepatic feature of AIH.
Medical records of patients with AIH and uveitis were reviewed retrospectively. One index case was identified from the Uveitis Clinic of the Casey Eye Institute of the Oregon Health & Science University. Further cases were identified by a web-based survey of members of the American Uveitis Society, the International Uveitis Study Group, the Proctor Foundation chat room and the SUN International Workshop. All respondents were sent a clinical data collection sheet requesting clinical information about uveitis phenotype, treatment and AIH features.
Clinical information was obtained for 9 individuals (4 females and 5 males; age range 20-70 years) who suffered from AIH and uveitis. The average duration of follow up was 49 months (range 11 months - 9 years). All patients had chronic, persistent, bilateral non-granulomatous uveitis that was anterior (n=3), intermediate (n=1) or pan (n=5) in location. In those with anterior uveitis, the onset was sudden, whereas the onset in the others was insidious. In 6 patients the diagnosis of uveitis preceded that of AIH by an average of 8 years (range <1 - 26 years), whereas the diagnosis of AIH preceded the diagnosis of uveitis by an average of 3 years (range 1 - 5 years) in 3 patients. Every patient had one or more complications arising from their uveitis. Cataract was the most common (N=7), followed by glaucoma (N=5), hypotony (N=4), cystoid macular edema (N=3), band keratopathy (N=3) and posterior synechiae (N=3). Less common were peripheral anterior synechiae (N=2), epiretinal membrane (N=1) and pthisis (N=1). Final visual acuities varied from 20/16 to light perception. To treat the uveitis and/or AIH, all patients required oral prednisone and 6 of 9 were treated with systemic immunosuppression.
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