Reappraisal of the management of Vogt–Koyanagi–Harada disease: sunset glow fundus is no more a fatality

• Published in: International ophthalmology Vol. 37; no. 6; pp. 1383 - 1395
• Main Authors: Herbort Jr, Carl P., Abu El Asrar, Ahmed M., Yamamoto, Joyce H., Pavésio, Carlos E., Gupta, Vishali, Khairallah, Moncef, Tugal-Tutkun, Ilknur, Soheilian, Masoud, Takeuchi, Masuru, Papadia, Marina
• Format: Journal Article
• Language: English
• Published: Dordrecht Springer Netherlands 01-12-2017; Springer Nature B.V
• Subjects: Adrenal Cortex Hormones - therapeutic use; Choroiditis; Corticosteroids; Disease control; Drug Administration Schedule; Fundus Oculi; Humans; Immunosuppressive agents; Immunosuppressive Agents - therapeutic use; Inflammation; Medical treatment; Medicine; Medicine & Public Health; Ophthalmology; Retinal Diseases - prevention & control; Review; Reviews; Stroma; Therapy; Uveomeningoencephalitic Syndrome - drug therapy; Indocyanine green angiography; Vogt–Koyanagi–Harada disease; Immunosuppressive therapy; Granulomatous uveitis; Stromal choroiditis, autoimmune disease
• ISSN: 0165-5701; 1573-2630
• DOI: 10.1007/s10792-016-0395-0

Purpose
Vogt–Koyanagi–Harada (VKH) disease is a primary autoimmune stromal choroiditis. Aim of the study was to gather a body of evidence from the literature and from experts that systemic corticosteroid combined with non-steroidal immunosuppressive therapy should become the standard of care in initial-onset VKH disease.
Methods
Literature was reviewed and leading experts in VKH were consulted in different parts of the world in order to put forward a consensus attitude in the management of initial-onset VKH disease.
Results
There was a substantial body of evidence in the literature that early aggressive and sustained corticosteroid and non-steroidal immunosuppressive therapy in initial-onset VKH disease allows to achieve full control of choroidal inflammation, eliminating any subclinical choroidal inflammation, and substantially reduces recurrences with improvement of anatomical and functional outcomes. This was in agreement with experts’ opinion and practice. ICGA was the method of choice to monitor disease evolution.
Conclusion
Since the choroidal space is easily accessible to systemic therapy and because inflammation in VKH disease is exclusively originating from the choroidal stroma, early and sustained treatment right at the onset of the disease process with dual corticosteroid and non-steroidal immunosuppressive therapy can result in full “healing” in many cases preventing sunset glow fundus which results from depigmentation from chronic uncontrolled inflammation.