Cracking The Code: Bilateral Optic Disc Swelling With Serous Retinal Detachment As A Clue Of Vogt-koyanagi-harada Disease
DOI:
https://doi.org/10.53366/jimki.v12i2.988Keywords:
Vogt-Koyanagi-Harada Disease, Optic Disc Swelling, Retinal Detachment, Corticosteroid.Abstract
Background: Vogt–Koyanagi–Harada disease (VKH) is a rare autoimmune disorder that attacks melanocyte-containing tissues, particularly the eyes, and can involve the central nervous system, hearing, and skin. VKH is one of the leading causes of non-infectious panuveitis in pigmented populations, and delayed diagnosis can lead to permanent visual impairment. Therefore, early detection and appropriate corticosteroid therapy are crucial to prevent long-term visual impairment. The purpose of this case report is to describe the clinical manifestations, supporting examinations, and response to high-dose corticosteroid therapy in a patient with suspected VKH disease. Case Illustration: A 39-year-old male presented with one week of progressive bilateral visual loss accompanied by fever, headache, and tinnitus. Examination revealed anterior chamber cells and flare, optic disc swelling, and serous retinal detachment. Optical coherence tomography confirmed subretinal fluid with septa formation. The patient was treated with high-dose intravenous methylprednisolone followed by oral tapering, resulting in significant visual improvement and stable recovery over one year. Conclusion: Early recognition of VKH disease and prompt initiation of high-dose corticosteroid therapy are crucial to control inflammation, prevent recurrences, and preserve long-term visual function.
References
1. Stern EM, Nataneli N. Vogt-Koyanagi-Harada Syndrome. StatPearls [Internet]. 2023 Apr 20 [cited 2025 Oct 1]
2. Herbort CP, Mochizuki M. Vogt-Koyanagi-Harada disease: inquiry into the genesis of a disease name in the historical context of Switzerland and Japan. Int Ophthalmol [Internet]. 2007;27(2–3):67–79.
3. Papasavvas I, Tugal-Tutkun I, Herbort CP. Vogt-Koyanagi-Harada is a Curable Autoimmune Disease: Early Diagnosis and Immediate Dual Steroidal and Non-Steroidal Immunosuppression are Crucial Prerequisites. J Curr Ophthalmol [Internet]. 2020;32(4):310–4.
4. Urzua CA, Herbort CP, Takeuchi M, Schlaen A, Concha-del-Rio LE, Usui Y, et al. Vogt-Koyanagi-Harada disease: the step-by-step approach to a better understanding of clinicopathology, immunopathology, diagnosis, and management: a brief review. J Ophthalmic Inflamm Infect. 2022;12(1):17.
5. Read RW, Holland GN, Rao NA, Tabbara KF, Ohno S, Arellanes-Garcia L, et al. Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: Report of an international committee on nomenclature. Am J Ophthalmol. 2001;131(5):647–52.
6. da Silva FTBGC, Damico FM, Marin ML, Goldberg AC, Hirata CE, Takiuti PH, et al. Revised diagnostic criteria for vogt-koyanagi-harada disease: considerations on the different disease categories. Am J Ophthalmol. 2009;147(2).
7. Baltmr A, Lightman S, Tomkins-Netzer O. Vogt-Koyanagi-Harada syndrome - current perspectives. Clin Ophthalmol. 2016;10:2345–61.
8. Tayal A, Daigavane S, Gupta N. Vogt-Koyanagi-Harada Disease: A Narrative Review. Cureus. 2024;16(4).
9. Lavezzo MM, Sakata VM, Morita C, Rodriguez EEC, Abdallah SF, Silva FTG Da, et al. Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes. Orphanet J Rare Dis. 2016;11(1).
10. Du L, Kijlstra A, Yang P. Vogt-Koyanagi-Harada disease: Novel insights into pathophysiology, diagnosis and treatment. Prog Retin Eye Res. 2016;52:84–111.
11. Diallo K, Revuz S, Clavel-Refregiers G, Sené T, Titah C, Gerfaud-Valentin M, et al. Vogt-Koyanagi-Harada disease: a retrospective and multicentric study of 41 patients. BMC Ophthalmol. 2020;20(1).
12. Kiyomoto C, Imaizumi M, Kimoto K, Abe H, Nakano S, Nakatsuka K. Vogt-Koyanagi-Harada disease in elderly Japanese patients. Int Ophthalmol. 2007;27(2–3):149–53.
13. Albaroudi N, Tijani M, Boutimzine N, Cherkaoui O. Clinical and therapeutic features of pediatric Vogt-Koyanagi-Harada disease. J Fr Ophtalmol. 2020;43(5):427–32.
14. Rutzen AR, Ortega-Larrocea G, Schwab IR, Rao NA. Simultaneous onset of Vogt-Koyanagi-Harada syndrome in monozygotic twins. Am J Ophthalmol. 1995;119(2):239–40.
15. Sugita S, Takase H, Kawaguchi T, Taguchi C, Mochizuki M. Cross-reaction between tyrosinase peptides and cytomegalovirus antigen by T cells from patients with Vogt-Koyanagi-Harada disease. Int Ophthalmol. 2007;27(2–3):87–95.
16. Prignano F, Betts CM, Lotti T. Vogt-Koyanagi-Harada disease and vitiligo: where does the illness begin? J Electron Microsc (Tokyo). 2008;57(1):25–31.
17. Yang P, Zhong Y, Du L, Chi W, Chen L, Zhang R, et al. Development and Evaluation of Diagnostic Criteria for Vogt-Koyanagi-Harada Disease. JAMA Ophthalmol. 2018;136(9):1025–31.
18. Ji H, Zhang N, Zhu M, Dong J, Jiang Z. Elevated Serum Immunoglobulin E Levels are Associated with the Severity of Newly Diagnosed, Acute Vogt-Koyanagi-Harada Disease. Curr Eye Res. 2022;47(1):102–6.
19. Balci O, Jeannin B, Herbort CP. Contribution of dual fluorescein and indocyanine green angiography to the appraisal of posterior involvement in birdshot retinochoroiditis and Vogt-Koyanagi-Harada disease. Int Ophthalmol. 2018;38(2):527–39.
20. Kim P, Sun HJ, Ham D Il. Ultra-wide-field angiography findings in acute Vogt-Koyanagi-Harada disease. Br J Ophthalmol. 2019;103(7):942–8.
21. Sugitani K, Hirano Y, Kurobe R, Hirahara S, Yasukawa T, Yoshida M, et al. Three-dimensional analysis of choroidal vessels in eyes with Vogt-Koyanagi-Harada disease before and after treatment. Can J Ophthalmol. 2020;55(6):500–8.
22. Fan S, Lin D, Hu J, Cao J, Wu K, Li Y, et al. Evaluation of microvasculature alterations in convalescent Vogt-Koyanagi-Harada disease using optical coherence tomography angiography. Eye (Lond). 2021;35(7):1993–8.
23. Verma S, Thakur H, Azad SV, Kumar V. Delayed-onset unilateral Vogt-Koyanagi-Harada syndrome: a multimodal imaging appraisal. BMJ Case Rep. 2021;14(2).
24. Aggarwal K, Agarwal A, Mahajan S, Invernizzi A, Mandadi SKR, Singh R, et al. The Role of Optical Coherence Tomography Angiography in the Diagnosis and Management of Acute Vogt-Koyanagi-Harada Disease. Ocul Immunol Inflamm. 2018;26(1):142–53.
25. Urzua CA, Velasquez V, Sabat P, Berger O, Ramirez S, Goecke A, et al. Earlier immunomodulatory treatment is associated with better visual outcomes in a subset of patients with Vogt-Koyanagi-Harada disease. Acta Ophthalmol.2015;93(6).
26. Heo JW, Cho BJ, Goldstein DA, Sepah YJ, Do D V, Nguyen QD. FLUOCINOLONE ACETONIDE IMPLANT FOR VOGT-KOYANAGI-HARADA DISEASE: Three-Year Outcomes of Efficacy and Safety. Retina. 2016;36(11):2124–31.
27. Park JG, Callaway NF, Ludwig CA, Mahajan VB. Intravitreal methotrexate and fluocinolone acetonide implantation for Vogt-Koyanagi-Harada uveitis. Am J Ophthalmol Case Reports.2020;19.
28. Takayama K, Obata H, Takeuchi M. Efficacy of Adalimumab for Chronic Vogt-Koyanagi-Harada Disease Refractory to Conventional Corticosteroids and Immunosuppressive Therapy and Complicated by Central Serous Chorioretinopathy. Ocul Immunol Inflamm. 2020;28(3):509–12.
29. Shen E, Rathinam SR, Babu M, Kanakath A, Thundikandy R, Lee SM, et al. Outcomes of Vogt-Koyanagi-Harada Disease: A Subanalysis From a Randomized Clinical Trial of Antimetabolite Therapies. Am J Ophthalmol. 2016;168:279–86.
30. Rathinam SR, Babu M, Thundikandy R, Kanakath A, Nardone N, Esterberg E, et al. A randomized clinical trial comparing methotrexate and mycophenolate mofetil for noninfectious uveitis. Ophthalmology. 2014;121(10):1863–70.
31. Arcinue CA, Radwan A, Lebanan MO, Foster CS. Comparison of two different combination immunosuppressive therapies in the treatment of Vogt-Koyonagi-Harada syndrome. Ocul Immunol Inflamm. 2013;21(1):47–52.
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Copyright (c) 2025 dr. Sani Ratna Saraswati, S.Ked, dr. I Ketut Aryawan, Sp.M
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