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      The incidence, presenting clinical findings and treatment patterns of Birdshot Retinochoroiditis in a high-prevalence region: findings from Northern Ireland, England and Wales

      1 , , 2 , 3 , 4 , 2 , 2 , 5
      Nature Publishing Group UK
      Epidemiology, Uveal diseases

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          Birdshot Retinochoroiditis (BSRC) is a rare, chronic posterior uveitis that is strongly associated with HLA-A*29.2 positivity. To date, no robust incidence studies of BSRC have been undertaken. We present the first epidemiological study of BSRC in a high-prevalence region.


          In collaboration with the British Ophthalmological Surveillance Unit, all new cases of BSRC between May 2017 and June 2019 were prospectively collected. Presenting demographics, symptoms, signs and treatment modalities were collected. A follow-up questionnaire twelve months later was also sent.


          Thirty-seven confirmed cases meeting the reporting criteria were identified. Twenty-three cases had both baseline and follow-up data. The total population incidence of BSRC was 0.035 cases per 100,000 person-years [95% CI 0.025–0.048 cases per 100 000 people]. 97.3% were HLA-A*29 positive. The median age was 46 years, with females making up 78% of patients. There were no significant differences in the latitudinal incidence of BSRC. At presentation, floaters were the most common symptom. Optic disc swelling was the most common sign. Mean presenting visual acuity was independent of symptom duration. Combined systemic corticosteroids and immunomodulatory therapy were the most common treatments at baseline and follow-up. Intravitreal steroids were equally popular at follow-up.


          This study provides the first nationwide estimate of the incidence of BSRC in a high-prevalence region. Cases were more common in females, with a broad range of presentation ages. No significant latitudinal effect of incidence was identified. Systemic therapy with steroids and IMT remain the most common treatments.

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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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            Visual acuities "hand motion" and "counting fingers" can be quantified with the freiburg visual acuity test.

            The visual acuity (VA) of patients with very low vision is classified using the semiquantitative scale "counting fingers" (CF), "hand motion" (HM), "light perception" (LP), and "no light perception." More quantitative measures would be desirable, especially for clinical studies. The results of clinical VA measurements, Early Treatment Diabetic Retinopathy Study (ETDRS) charts, and the Freiburg Visual Acuity Test (FrACT) were compared. The FrACT is a computerized visual acuity test that can present very large Landolt C optotypes when necessary. Examined were 100 eyes of 100 patients with various eye diseases (e.g., diabetic retinopathy, ARMD), covering a range of VAs from LP to decimal 0.32. The FrACT optotypes were presented on a 17-inch LCD monitor with random orientation. After extensive training, two ETDRS and FrACT measurements were obtained. The testing distance was 50 or 100 cm. ETDRS and FrACT coincided closely for VA > or = 0.02 (n = 80). ETDRS measures were successfully obtainable down to CF (at 30 cm; test-retest averaged over all patients, coefficient of variation [CV](ETDRS) = 9% +/- 8%), and FrACT provided reproducible measurements down to HM (test-retest CV(FrACT) =12% +/- 11%). For CF (n = 6), both ETDRS and FrACT resulted in a mean VA of 0.014 +/- 0.003 (range, 0.01-0.02). The VA results of FrACT for HM (n = 12) were 0.005 +/- 0.002 (range, 0.003-0.009); the individual values were highly reproducible. No results were obtainable for LP (n = 2). The three acuity procedures concur above a VA of 0.02. The results suggest that the category CF at 30 cm can be replaced by 0.014, using ETDRS or FrACT. Using FrACT, one can even reproducibly quantify VA in the HM-range, yielding a mean VA of 0.005.
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              Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel.

              To provide recommendations for the use of immunosuppressive drugs in the treatment of patients with ocular inflammatory disorders. A 12-person panel of physicians with expertise in ophthalmologic, pediatric, and rheumatologic disease, in research, and in the use of immunosuppressive drugs in patient care. Published clinical study results. Recommendations were rated according to the quality and strength of available evidence. The panel was convened in September of 1999 and met regularly through May 2000. Subgroups of the panel summarized and presented available information on specific topics to the full panel; recommendations and ratings were determined by group consensus. Although corticosteroids represent one of the mainstays in the management of patients with ocular inflammation, in many patients, the severity of the disease, the presence of corticosteroid side effects, or the requirement for doses of systemic corticosteroids highly likely to result in corticosteroid complications supports the rationale for immunosuppressive drugs (for example, antimetabolites, T-cell inhibitors, and alkylating agents) being used in the management of these patients. Because of the potential for side effects, treatment must be individualized and regular monitoring performed. With careful use of immunosuppressive drugs for treatment of ocular inflammatory disorders, many patients will benefit from them either with better control of the ocular inflammation or with a decrease in corticosteroid side effects.

                Author and article information

                Eye (Lond)
                Eye (Lond)
                Nature Publishing Group UK (London )
                10 February 2023
                10 February 2023
                September 2023
                : 37
                : 13
                : 2817-2825
                [1 ]GRID grid.4912.e, ISNI 0000 0004 0488 7120, Royal College of Surgeons in Ireland, ; 123 St Stephen’s Green, Dublin, D02 YN77 Republic of Ireland
                [2 ]GRID grid.436474.6, ISNI 0000 0000 9168 0080, Moorfields Eye Hospital NHS Foundation Trust, ; 162 City Road, London, EC1V 2PD United Kingdom
                [3 ]GRID grid.420468.c, Great Ormond Street Hospital for Children, Great Ormond Street, ; London, WC1N 3JH United Kingdom
                [4 ]GRID grid.83440.3b, ISNI 0000000121901201, UCL Institute of Ophthalmology, ; 11-43 Bath St, London, EC1V 9EL United Kingdom
                [5 ]GRID grid.4868.2, ISNI 0000 0001 2171 1133, Queen Mary University of London, ; Mile End Rd, Bethnal Green, London, E1 4NS United Kingdom
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                © The Author(s) 2023

                Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

                : 20 June 2022
                : 10 January 2023
                : 23 January 2023
                Funded by: FundRef https://doi.org/10.13039/501100000615, Fight for Sight UK;
                Award ID: 24BU152
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                © The Royal College of Ophthalmologists 2023

                Vision sciences
                epidemiology,uveal diseases
                Vision sciences
                epidemiology, uveal diseases


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