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      Uveitis- a rare disease often associated with systemic diseases and infections- a systematic review of 2619 patients


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          Uveitis is an autoimmune disease of the eye that refers to any of a number of intraocular inflammatory conditions. Because it is a rare disease, uveitis is often overlooked, and the possible associations between uveitis and extra-ocular disease manifestations are not well known. The aim of this study was to characterize uveitis in a large sample of patients and to evaluate the relationship between uveitis and systemic diseases.


          The present study is a cross-sectional study of a cohort of patients with uveitis. Records from consecutive uveitis patients who were seen by the Uveitis Service in the Department of Ophthalmology at the Medical University of Vienna between 1995 and 2009 were selected from the clinical databases. The cases were classified according to the Standardization of Uveitis Nomenclature Study Group criteria for Uveitis.


          Data were available for 2619 patients, of whom 59.9% suffered from anterior, 14.8% from intermediate, 18.3% from posterior and 7.0% from panuveitis. 37.2% of all cases showed an association between uveitis and extra-organ diseases; diseases with primarily arthritic manifestations were seen in 10.1% of all cases, non-infectious systemic diseases (i.e., Behçet´s disease, sarcoidosis or multiple sclerosis) in 8.4% and infectious uveitis in 18.7%. 49.4% of subjects suffering from anterior uveitis tested positively for the HLA-B27 antigen. In posterior uveitis cases 29% were caused by ocular toxoplasmosis and 17.7% by multifocal choroiditis.


          Ophthalmologists, rheumatologists, infectiologists, neurologists and general practitioners should be familiar with the differential diagnosis of uveitis. A better interdisciplinary approach could help in tailoring of the work-up, earlier diagnosis of co-existing diseases and management of uveitis patients.

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          Most cited references32

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          Incidence and prevalence of uveitis in Northern California; the Northern California Epidemiology of Uveitis Study.

          To determine the incidence and prevalence of uveitis in a large, well-defined population in Northern California. Cross-sectional study using retrospective database and medical record review. A group of 2070 people within 6 Northern California medical center communities (N = 731 898) who had a potential diagnosis of uveitis. The patient database of a large health maintenance organization (2 805 443 members at time of the study) was searched for all patients who, during a 12-month period, had the potential diagnosis of uveitis. Detailed quarterly gender- and age-stratified population data were available. Medical records of patients who potentially had uveitis and who were members of the 6 target communities were reviewed by 2 uveitis subspecialists to confirm the diagnosis of uveitis and to establish time of onset. Demographic and clinical data were gathered for patients meeting the clinical definition of uveitis. Incidence rates were calculated by using a dynamic population model. Prevalence rates were based on the mid-study period population. Presence and date of onset of uveitis. At midstudy, the population for the 6 communities was 731 898. During the target period, 382 new cases of uveitis were diagnosed; 462 cases of uveitis were diagnosed before the target period. These data yielded an incidence of 52.4/100 000 person-years and a period prevalence of 115.3/100 000 persons. The incidence and prevalence of disease were lowest in pediatric age groups and were highest in patients 65 years or older (P<0.0001). The prevalence of uveitis was higher in women than in men (P<0.001), but the difference in incidence between men and women was not statistically significant. Comparison between the group of patients who had onset of uveitis before the target period (ongoing uveitis) and the entire cohort of uveitis patients showed that women had a higher prevalence of ongoing uveitis than men and that this difference was largest in the older age groups (P<0.001). In this largest population-based uveitis study in the United States to date, the incidence of uveitis was approximately 3 times that of previous U.S. estimates and increased with the increasing age of patients. Women had a higher prevalence of uveitis than men, and the largest differences were in older age groups.
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            Causes and frequency of blindness in patients with intraocular inflammatory disease.

            Uveitis, an intraocular inflammatory disease, is a significant cause of visual impairment. It is not known how many patients with uveitis will retain visual acuity and how many develop visual impairment or even blindness. The aim of this study was to assess the frequency of blindness in patients with uveitis and, more specifically, to identify the clinical profile of patients at risk for visual loss. A cross sectional and retrospective study of 582 patients with uveitis who visited the ophthalmology departments of two university hospitals in the Netherlands was performed. Within the group of 582 patients, 203 (35%) exhibited blindness or visual impairment; bilateral legal blindness developed in 22 (4%) patients, 26 (4.5%) had one blind eye with visual impairment of the other, and nine (1.5%) had bilateral visual impairment. Unilateral blindness developed in 82 (14%) patients, whereas 64 (11%) exhibited unilateral visual impairment. The most important cause of both blindness and visual impairment was cystoid macular oedema (29% and 41%, respectively). Complications of uveitis were encountered in more than half of the patients and 23% underwent one or more surgical procedures. When the patients were subdivided according to anatomical site, those with panuveitis had the worst visual prognosis. The systemic diseases associated with a poor visual prognosis were juvenile chronic arthritis and sarcoidosis. Ocular toxoplasmosis was the most frequent cause of unilateral visual loss. Cystoid macular oedema is the most frequent complication of uveitis and its occurrence plays a decisive role in the visual outcome of this disease.
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              The possible impact of uveitis in blindness: a literature survey.


                Author and article information

                Orphanet J Rare Dis
                Orphanet J Rare Dis
                Orphanet Journal of Rare Diseases
                BioMed Central
                29 August 2012
                : 7
                : 57
                [1 ]Department of Ophthalmology and Optometry, Medical University Vienna, Waehringer Guertel 18-20, A-1090, Vienna, Austria
                [2 ]Laura Bassi Centre for Ocular Inflammation and Infection, Institute for Specific Prophylaxis and Tropical Medicine, Medical University Vienna, Kinderspitalgasse 15, A-1090, Vienna, Austria
                [3 ]Department of Ophthalmology, Hietzing Hospital, Wolkersbergenstrasse 1, A-1130, Vienna, Austria
                [4 ]Department of Pediatrics and adolescent medicine. Division of Pediatric Nephrology and Gastroenterology, Medical University Vienna, Waehringer Guertel 18-20, A-1090, Vienna, Austria
                [5 ]Department of Rheumatology, Medical University Vienna, Waehringer Guertel 18-20, A-1090, Vienna, Austria
                [6 ]Department of Medical Parasitology, Institute for Specific Prophylaxis and Tropical Medicine, Medical University Vienna, Waehringer Guertel 18-20, A-1090, Vienna, Austria
                Copyright ©2012 Barisani-Asenbauer et al.; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                : 21 February 2012
                : 13 August 2012

                Infectious disease & Microbiology
                systemic associations,infections,uveitis,arthritis,etiology
                Infectious disease & Microbiology
                systemic associations, infections, uveitis, arthritis, etiology


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