Reactivation of Vogt-Koyanagi-Harada disease under control for more than 6 years, following anti-SARS-CoV-2 vaccination

Abstract

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.

Keywords: Anti-SARS-CoV-2 vaccination; Autoimmune stromal choroiditis; Vogt-Koyanagi-Harada disease: Indocyanine green angiography.

Fig. 1

Case of VKH at presentation. ICGA picture ODS shows exudative detachments and numerous HDDs. OCT shows retinal folds and subretinal fluid and very thickened choroids impossible to measure on EDI-OCT

Fig. 2

FA: exsudative retinal detachements around optic disc (a) and temporal to the fovea OS (b)

Fig. 3

ICGA ODS of VKH patient at the time of prednisone discontinuation. ICGA shows re-apparition of HDDs at the moment of prednisone discontinuation, despite mycophenolic acid and cyclosporine therapy that led to the introduction of infliximab treatment

Fig. 4

a ICGA and OCT after 6 years of infliximab treatment. ICGA pictures show a normal choroid devoid of HDDs and normal OCT images ODS (insert). b Fundus photographs ODS seven years after initial-onset VKH disease. Absence of notable sunset glow fundus 2 months prior to post-vaccination recurrence

Fig. 5

Recurrence of VKH disease after 6 inflammation free years. While the initial-onset disease was non-granulomatous, mutton-fat KPs were seen during post-vaccine recurrence OD (yellow arrows)

Fig. 6

Recurrence of VKH after the second dose of anti-SARS-CoV-2 vaccination. On ICGA, numerous HDDs ODS dispersed over both fundi are present. OCT shows exudative retinal detachments and markedly thickened choroid not measurable OCT OD1 and OCT OS1. Thickness decreased substantially after 5 days of oral corticosteroids to 506 ± 50 μm OD and 454 ± 28 μm OG (OCT OD 2 & OCT OD 2)