Abstract
Purpose: :
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.
Methods: :
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.
Results: :
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.
Keywords: autoimmune disease • uveitis-clinical/animal model • inflammation