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      Update on Sympathetic Ophthalmia

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          Abstract

          Sympathetic ophthalmia (SO) is a bilateral diffuse granulomatous intraocular inflammation that occurs in most cases within days or months after surgery or penetrating trauma to one eye. The incidence of SO ranges from 0.2 to 0.5% after penetrating ocular injuries and 0.01% after intraocular surgery. Vitreoretinal surgery and cyclodestructive procedures are considered risk factors. The time from ocular injury to onset of SO varies greatly, ranging from a few days to decades, with 80% of the cases occurring within 3 months after injury to the exciting eye and 90% within 1 year. The diagnosis is based on clinical findings rather than on serological testing or pathological studies. It presents as a bilateral diffuse uveitis. Patients report an insidious onset of blurry vision, pain, epiphora, and photophobia in the sympathizing, non-injured eye. Classically this is accompanied by conjunctival injection and a granulomatous anterior chamber reaction with mutton-fat keratic precipitates (KPs) on the corneal endothelium. In the posterior segment, the extent of inflammation can vary. Systemic corticosteroids are the first line therapy for SO. If patients are non-responsive to steroid therapy or have clinically significant side effects, cyclosporine, azathioprine or other immunosuppressive agents can be used for long-term immunomodulatory therapy.

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          Most cited references 56

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          The characteristic features of optical coherence tomography in posterior uveitis.

          To describe the different retinal morphological characteristics that can present on optical coherence topography (OCT) in a spectrum of uveitic diseases. We reviewed the literature and our own OCT image archive for characteristic features that may be suggestive of a particular disease process. OCT demonstrates a variety of characteristic morphological changes, some that may point towards a specific disease process. We describe the various forms of macular oedema found in uveitis as well as OCT features typically found in multifocal choroiditis, serpiginous chorioretinitis, toxoplasma chorioretinitis, Vogt-Koyanagi-Harada, sympathetic ophthalmia and the vitreomacular traction syndrome. Ophthalmologists should be aware of the variety of retinal morphological characteristics that can present on OCT in uveitic disease. Recognition may aid in the diagnostic process, which is complementary to conventional fundal photography and fluorescein angiography. This can facilitate earlier diagnosis and, more importantly, the initiation of specific treatment.
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            Prospective surveillance of sympathetic ophthalmia in the UK and Republic of Ireland.

            To establish current epidemiological data, risks, and interventional outcomes of newly diagnosed sympathetic ophthalmia (SO). Prospective surveillance took place of all permanently employed ophthalmologists in the UK and Republic of Ireland by a monthly reporting card through the British Ophthalmological Surveillance Unit. Case ascertainment was made of newly diagnosed SO from July 1997 and questionnaire data were returned at baseline, 6 months, and 1 year after diagnosis. 23 patients with newly diagnosed SO were recruited over 15 months, corresponding to a minimum estimated incidence of 0.03/100 000. Baseline data were available on 18 patients, in whom SO occurred after surgery in 11 patients, after retinal surgery alone in six patients, and after accidental trauma in seven patients. 12 of the 16 patients with 1 year follow up had a visual acuity of 6/12 or better. Good visual outcome was related to prompt and adequate systemic immunosuppressive therapy. The incidence of sympathetic ophthalmia is very low. The main current risk is surgery, particularly retinal surgery, but visual prognosis is good if early diagnosis is made and rapid, adequate immunotherapy is commenced.
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              HLA associations and ancestry in Vogt-Koyanagi-Harada disease and sympathetic ophthalmia.

              A strong association with HLA antigens DR4, DRw53, and Bw54 has previously been reported among Japanese patients with Vogt-Koyanagi-Harada disease (VKH) and sympathetic ophthalmia (SO). In the United States, no firm association between HLA-A or -B loci and VKH has been found previously; testing for HLA-DR loci has not been performed to date. The authors performed HLA typing of 23 American patients with VKH and 8 patients with SO. When VKH patients were compared with racially matched controls without disease and patients with other types of uveitis, strong associations with HLA-DR4 and HLA-DRw53 were found. The strongest associations observed in this sample were with HLA-DQw3, an antigen which is in positive linkage disequilibrium with DR4, and with the HLA-DR4/DQw3 haplotype. The small number of patients with SO precluded statistical analysis; however, similar HLA associations were noted. The patients also were questioned regarding their ancestry. The anecdotal association of VKH with American Indian ancestry was confirmed. It appears that the ethnoracial association may be explained by HLA type. One possible explanation for identical HLA associations in two diseases with different precipitating events yet similar ocular manifestations is development of an altered immune response to exogenous microbial antigen with subsequent autoimmunity. Further definition of the genetic susceptibility to VKH and SO may help define the pathophysiology of both diseases and allow the prediction of which patients are at increased risk for SO.
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                Author and article information

                Journal
                Middle East Afr J Ophthalmol
                MEAJO
                Middle East African Journal of Ophthalmology
                Medknow Publications & Media Pvt Ltd (India )
                0974-9233
                0975-1599
                Jan-Mar 2012
                : 19
                : 1
                : 13-21
                Affiliations
                [1 ]Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD, USA
                [2 ]The Vitreo-Retinal and Uveitis Division, King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia
                [3 ]The Clínica Oftalmológica El Viñedo, Valencia, Venezuela
                [4 ]The Retina and Vitreous Service, Clínica Oftalmológica Centro Caracas, and the Arevalo-Coutinho Foundation for Research in Ophthalmology, Caracas, Venezuela
                Author notes
                Corresponding Author: Dr. J. Fernando Arevalo, Vitreoretinal Division, The King Khaled Eye Specialist Hospital, Al-Oruba Street, PO Box 7191, Riyadh 11462, Kingdom of Saudi Arabia. E-mail: arevalojf@ 123456jhmi.edu
                Article
                MEAJO-19-13
                10.4103/0974-9233.92111
                3277011
                22346110
                Copyright: © Middle East African Journal of Ophthalmology

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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