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DOI: 10.1055/a-2795-6069
Birdshot Chorioretinopathy with Cystoid Macular Oedema
Birdshot-Chorioretinopathie mit zystoidem MakulaödemAuthors
Abstract
We report the case of a 48-year-old Caucasian male who presented with progressive bilateral visual decline over more than three months. He was diagnosed with birdshot chorioretinopathy (BSCR) according to the Standardization of Uveitis Nomenclature (SUN) criteria, based on characteristic fundus findings and HLA-A29 positivity. Laboratory investigations, including angiotensin-converting enzyme (ACE) and soluble IL-2 receptor levels, were within normal limits and infectious serologies were negative. Thoracic imaging by computed tomography showed no signs suggestive of sarcoidosis or infection, and pulmonary function testing was normal. Systemic corticosteroids combined with methotrexate improved vitritis but left cystoid macular edema (CME) largely unchanged, especially in the right eye. Therapy was therefore switched to mycophenolate mofetil and adalimumab, which produced a marked reduction of CME, although some cystic changes persisted. This case highlights the therapeutic challenges in the real-world management of BSCR complicated by persistent CME. Even with reduction of intraocular inflammation, CME may remain resistant to systemic corticosteroids alone, particularly in patients presenting late in the disease course. Early diagnosis, exclusion of systemic mimics, and a flexible, imaging-guided approach to treatment are essential for optimising visual prognosis.
Publication History
Received: 02 November 2025
Accepted: 22 January 2026
Article published online:
23 February 2026
© 2026. Thieme. All rights reserved.
Georg Thieme Verlag KG
Oswald-Hesse-Straße 14, 70469 Stuttgart, Germany
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References
- 1 Pichi F, Miserocchi E, Grewal DS. et al. Evidence and Consensus-based Imaging Guidelines in Birdshot Chorioretinopathy: Multimodal Imaging in Uveitis (MUV) Taskforce Report 8. Am J Ophthalmol 2025; 278: 271-281
- 2 Bousquet E, Duraffour P, Debillon L. et al. Birdshot Chorioretinopathy: A Review. J Clin Med 2022; 11: 4772
- 3 Standardization of Uveitis Nomenclature (SUN) Working Group. Classification Criteria for Birdshot Chorioretinitis. Am J Ophthalmol 2021; 228: 65-71
- 4 Kuiper J, Rothova A, de Boer J. et al. The immunopathogenesis of birdshot chorioretinopathy; a bird of many feathers. Prog Retin Eye Res 2015; 44: 99-110
- 5 Gelfman S, Monnet D, Ligocki AJ. et al. ERAP1, ERAP2, and Two Copies of HLA-Aw19 Alleles Increase the Risk for Birdshot Chorioretinopathy in HLA-A29 Carriers. Invest Ophthalmol Vis Sci 2021; 62: 3
- 6 Calvo-Río V, Blanco R, Santos-Gómez M. et al. Efficacy of Anti-IL6-Receptor Tocilizumab in Refractory Cystoid Macular Edema of Birdshot Retinochoroidopathy Report of Two Cases and Literature Review. Ocul Immunol Inflamm 2017; 25: 604-609
- 7 Doycheva D, Jägle H, Zierhut M. et al. Mycophenolic acid in the treatment of birdshot chorioretinopathy: long-term follow-up. Br J Ophthalmol 2015; 99: 87-91
- 8 Ferreira de Moura T, Enjalbert A, Monnet D. et al. Peripapillary atrophy in patients with birdshot chorioretinitis. Br J Ophthalmol 2025; 109: e326440
- 9 Ferracci D, Mathis T, Gavoille A. et al. Long-Term Outcomes of Birdshot Chorioretinopathy Treated with Corticosteroids: A Case Reports. J Clin Med 2023; 12: 5288
- 10 Menezo V, Taylor SRJ. Birdshot uveitis: current and emerging treatment options. Clin Ophthalmol 2013; 8: 73
