Vogt–Koyanagi–Harada (VKH) disease is a primary autoimmune granulomatous choroiditis that begins in the choroidal stroma. The aim of this review was to gather a body of evidence for the concept of a window of therapeutic opportunity, defined as a time interval following initial-onset disease during which adequate treatment will substantially modify the disease outcome and possibly even lead to cure, similar to what has been described for rheumatoid arthritis.

Birdshot retinochoroiditis (BRC) is a uveitis predominantly affecting the posterior segment of the eye with dual, independent retinal and choroidal inflammation and almost only seen in Caucasians.1 , 2 Ryan and Maumenee first described the bilateral retinochoroidal inflammatory disease in early 1980, reporting on 13 patients.2 These authors called it ‘birdshot retinochoroidopathy’ because they found that lesions had a pattern comparable to that seen in wounds produced by birdshot shotguns. Later in 1980, Kaplan and Aaberg published a series of four similar cases ‘involving a choroidal and retinal pigment epithelium disease not previously described’, which they also called birdshot retinochoroidopathy.3 In 1981, Gass described 11 similar cases and called the disease vitiliginous chorioretinitis.4

Background/purpose: Vogt-Koyanagi-Harada (VKH) disease is a primary stromal choroiditis with bilateral granulomatous panuveitis. If initial-onset VKH is treated early and relentlessly the disease can be controlled and even “cured” in a substantial number of cases. We are reporting on a patient treated early and in a sustained fashion who was inflammation free for seven years but who presented a reactivation 6 weeks after the second dose of anti-SARS-CoV-2 vaccination. Case report: A 43-year-old woman presented with severe initial-onset VKH disease which was brought under control using steroidal and non-steroidal Immunosuppression (mycophenolic acid and cyclosporine) with additional infliximab infusions because of the persistence of subclinical choroiditis identified on ICGA. Under infliximab alone disease had been inflammation free with no subclinical disease and absence of sunset glow fundus for 6 years. However, following anti-SARS-CoV-2 vaccination, severe resurgence of the disease occurred with exudative retinal detachments. Disease was rapidly brought again under control with oral prednisone (1 mg/kg) therapy and a new loading scheme of infliximab therapy. Conclusion: VKH disease results from an autoimmune process directed against melanocyte associated antigens which can be controlled when early and sustained immunosuppressive treatment is introduced. It seems that anti-SARS-CoV-2 vaccination can be at the origin of reactivation of long-time controlled disease.

Background: Multiple evanescent white dot syndrome (MEWDS) is a rare inflammatory eye condition affecting the outer retina as a consequence of choriocapillaris non perfusion. The pathophysiology of MEWDS will be discussed based clinical appraisal and on multimodal imaging appraisal. Methods: Narrative review and perspective opinion. Results: Literature review results helped us to put forward (1) the specific symptomatology (decreased/blurred vision, photopsia, subjective scotomas), (2) the ill-asserted character of clinical findings (foveal granularity, white dots in fundoscopy), (3) and the crucial importance of multimodal imaging with the diagnostic triad of ICGA hypofluorescent areas, BL-FAF hyperautofluorescent areas and loss/damage of IS/OS-ellipsoid zone on SD-OCT that characterise the disease and can practically help the clinician to diagnose MEWDS. A comprehensive alternative perspective of the disease was formulated. Conclusions: The bulk of evidence that we are presenting in this review, thanks to new performing non-invasive and invasive imaging modalities, is sufficiently compelling to consider MEWDS as a primary choriocapillaritis/inflammatory choriocapillaropathy. Multimodal imaging allows the clinician to diagnose MEWDS with a high level of certainty and ensures a precise follow-up.

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.