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Vogt–Koyanagi–Harada disease: recurrence rates after initial-onset disease differ according to treatment modality and geographic area

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Abstract

Background/Purpose Vogt–Koyanagi–Harada (VKH) disease is a primary autoimmune stromal choroiditis producing a spill-over panuveitis. For initial-onset VKH disease, it is increasingly thought that corticosteroid therapy is not sufficient and additional non-steroidal immunosuppressive therapy is needed. At the 11th workshop on VKH, the disease was said to be well controlled with corticosteroids alone in Japanese patients. The aim of this study was to review the literature to determine whether different levels of severity exist in different geographical areas.

Methods

Literature was reviewed for studies on the evolution of initial-onset VKH disease, looking at treatment modalities and proportion of cases with chronic evolution and/or sunset-glow fundus (SGF).

Results

PubMed search yielded 1249 references containing the term of Vogt–Koyanagi–Harada. Twenty references (15 from outside of Japan and 5 from Japan) contained information on the evolution of treated initial-onset disease. For the “international” group, percentage of chronic evolution after systemic corticosteroid monotherapy was 61%, and after combined steroidal and non-steroidal therapy it fell to 2% (0% in 3/4 studies). In the Japanese studies where all patients received systemic corticosteroids alone, chronic evolution was reported in 25%; however, SGF amounted to 61%.

Conclusion

In the world at large, chronic evolution of initial-onset VKH disease treated with corticosteroids alone concerned two-thirds of patients. Japanese studies showed that chronic evolution was substantially less frequent, indicating possibly less severe disease in Japan. This proportion fell to almost zero when dual steroidal and non-steroidal immunosuppression was given at onset.

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Acknowledgements

All procedures performed in studies involving human participants were in accordance with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Authors and Affiliations

  1. Retinal and Inflammatory Eye Diseases, Centre for Ophthalmic Specialized Care (COS), Clinic Montchoisi Teaching Centre, Rue Charles-Monnard 6, CH-1003, Lausanne, Switzerland

    Carl P. Herbort Jr.

  2. Department of Ophthalmology, University of Lausanne, Lausanne, Switzerland

    Carl P. Herbort Jr.

  3. Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

    Ilknur Tugal-Tutkun

  4. Department of Ophthalmology, Faculty of Medicine, Fattouma Bourguiba University Hospital, University of Monastir, Monastir, Tunisia

    Moncef Khairallah

  5. Department of Ophthalmology, Dr. Nasser Al-Rashid Research Chair in Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia

    Ahmed M. Abu el Asrar

  6. National Institute for Health Research, Biomedical Research Centre at Moorfields Eye Hospital, NHS Foundation Trust and UCL, Institute of Ophthalmology, London, UK

    Carlos E. Pavésio

  7. Department of Ophthalmology and Ophthalmic Research Centre, Labbafinejad Medical Centre, Shahid Beheshti Medical University, Teheran, Iran

    Masoud Soheilian

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Herbort, C.P., Tugal-Tutkun, I., Khairallah, M. et al. Vogt–Koyanagi–Harada disease: recurrence rates after initial-onset disease differ according to treatment modality and geographic area. Int Ophthalmol 40, 2423–2433 (2020). https://doi.org/10.1007/s10792-020-01417-1

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