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Background <p>Vogt–Koyanagi–Harada (VKH) disease is a&#xa0;rare, multisystemic autoimmune disease that affects melanin-containing tissues such as the eye, the inner ear, the meninges, and the skin. The typical ocular manifestation is bilateral granulomatous panuveitis with serous retinal detachments. The diagnosis is primarily made clinically using optical coherence tomography (OCT) and angiography. Systemic corticosteroids are used as the initial therapy, and immunosuppressive drugs are used for long-term treatment, if necessary.</p> Materials and methods <p>In this retrospective case series, we present the clinical courses and treatments of all patients with VKH disease (<i>n</i> = 9) who first presented at the Department of Ophthalmology at the Medical University of Graz between the years 2006 and 2022.</p> Results <p>The average age of patients was 37&#xa0;years, with women affected more than three times as often as men (ratio 7:2). All patients reported visual deterioration at first presentation, 6&#xa0;patients experienced headaches, and 2&#xa0;patients had auditory symptoms. Initial treatment consisted of oral corticosteroids (50–100 mg/day) in 7&#xa0;patients, with 2&#xa0;patients initially receiving intravenous corticosteroids. Recurrences occurred in 5&#xa0;cases; immunosuppressive therapy was added in 5&#xa0;patients. Two patients experienced multiple recurrences and were ultimately treated with Tumor necrosis factor (TNF)-α blockers.</p> Conclusion <p>The results of this retrospective case series underscore the challenges in the treatment of VKH disease. The high rate of recurrences and the need for supplementary immunosuppressive drugs in many cases highlight the importance of long-term supervision and adaptation of treatment tailored to the individual requirements of patients. The use of TNF‑α blockers in recurrent cases seems to be a&#xa0;promising option for long-term treatment.</p>

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Therapien und klinische Verläufe des Vogt-Koyanagi-Harada-Syndroms an einem tertiären Versorgungszentrum in Österreich: eine retrospektive Fallserie

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  • 发表日期：

    2026年4月22日

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  • Tobias Peschaut,
  • G. Seidel,
  • M. Sommer,
  • M. Kruger,
  • L. Haiden,
  • D. Szabo,
  • P. Werkl

摘要

Background

Vogt–Koyanagi–Harada (VKH) disease is a rare, multisystemic autoimmune disease that affects melanin-containing tissues such as the eye, the inner ear, the meninges, and the skin. The typical ocular manifestation is bilateral granulomatous panuveitis with serous retinal detachments. The diagnosis is primarily made clinically using optical coherence tomography (OCT) and angiography. Systemic corticosteroids are used as the initial therapy, and immunosuppressive drugs are used for long-term treatment, if necessary.

Materials and methods

In this retrospective case series, we present the clinical courses and treatments of all patients with VKH disease (n = 9) who first presented at the Department of Ophthalmology at the Medical University of Graz between the years 2006 and 2022.

Results

The average age of patients was 37 years, with women affected more than three times as often as men (ratio 7:2). All patients reported visual deterioration at first presentation, 6 patients experienced headaches, and 2 patients had auditory symptoms. Initial treatment consisted of oral corticosteroids (50–100 mg/day) in 7 patients, with 2 patients initially receiving intravenous corticosteroids. Recurrences occurred in 5 cases; immunosuppressive therapy was added in 5 patients. Two patients experienced multiple recurrences and were ultimately treated with Tumor necrosis factor (TNF)-α blockers.

Conclusion

The results of this retrospective case series underscore the challenges in the treatment of VKH disease. The high rate of recurrences and the need for supplementary immunosuppressive drugs in many cases highlight the importance of long-term supervision and adaptation of treatment tailored to the individual requirements of patients. The use of TNF‑α blockers in recurrent cases seems to be a promising option for long-term treatment.