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

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.

Melanin change of retinal pigment epithelium and choroid in the convalescent stage of Vogt-Koyanagi-Harada disease

AIM: To observe the melanin change of the retinal pigment epithelium (RPE) and choroid in the convalescent stage of Vogt-Koyanagi-Harada (VKH). METHODS: A retrospective study was performed on 40 eyes of 20 patients in the convalescent stage of VKH. Fundus photography (FP), multi-spectral imaging (MSI), and optical coherence tomography (OCT) were performed. RESULTS: In the VKH convalescent stage, focal RPE melanin accumulation (FRMA) was detected in 34 eyes (85%) on MSI and in 7 eyes (17.5%) on FP. FRMA was limited to the previous retinal detachment area in all 28 eyes (FRMA was detected in 34 eyes on MSI, which were enrolled, and 6 eyes lacked data in the acute stage). Sunset-glow fundus was detected in 20 eyes (50%) on FP. The mean density of FRMA in a 1-mm-diameter circular area of the fovea was 0.04±0.07 on MSI, which was significantly correlated with sunset-glow fundus (ρ=0.467, P=0.02). CONCLUSION: In the VKH convalescent stage, FRMA is derived from the RPE melanin change, and sunset-glow fundus is derived from the choroid melanin change. A higher density of FRMA in the fovea and sunset-glow fundus represents more serious depigmentation of melanin.

Identification of Underlying Inflammation in Vogt–Koyanagi–Harada Disease with Sunset Glow Fundus by Multiple Analyses

Purpose. To evaluate underlying subclinical ocular inflammation in Vogt–Koyanagi–Harada (VKH) disease with sunset glow fundus (SGF) by multiple analyses. Study Design. Retrospective observational study. Methods. Clinical records of 34 eyes of 17 VKH patients with SGF in whom laser flare photometry (LFP), enhanced depth imaging optical coherence tomography (EDI-OCT), and indocyanine green angiography (ICGA) were performed on the same day were reviewed. The mean age was 57.3 ± 16.3 years, and the mean duration from the initial onset of uveitis was 47.1 ± 22.1 months. Flare counts, ICGA scores, and subfoveal choroidal thickness (SFCT) were compared between eyes. Results. Although clinical ocular inflammation was observed only in 4 eyes (11.8%), inflammatory signs were observed in 23 out of 34 eyes by LFP (67.6%), in 27 eyes by ICGA (79.4%), and in 10 eyes by SFCT (29.4%). Active inflammatory signs detected by ICGA were observed in 77.8% by LFP and in 25.9% by SFCT. The strength of agreement (Cohen’s kappa coefficient) between positive ICGA score and positive flare score was 0.406 (95% CI: 0.076–0.7359, P<0.01), but there was no association between positive ICGA score and increased SFCT. In addition, positive flare count was the significant prognostic factor of positive ICGA score with odds ratio 11.7. Conclusions. Subclinical ocular inflammation signs were detected in most VKH patients with SGF by ICGA and a substantial proportion of which were also detected by LFP, whereas SFCT was less sensitive to detect subclinical inflammation.

Retinal vessel oxygen saturation is affected in uveitis associated with Vogt-Koyanagi-Harada disease

AimsTo discover whether retinal vessel oxygen metabolism is affected in uveitis associated with Vogt-Koyanagi-Harada (VKH) disease.Methods41 patients with VKH disease (82 eyes) and 12 healthy subjects (24 eyes) matched in age and gender were prospectively evaluated. Retinal oxygen saturation and vessel calibre were measured with a non-invasive spectrophotometric retinal oximeter (Oxymap T1).ResultsIn healthy controls, mean arteriolar oxygen saturation (%) was 93.8±5.9 and venular saturation was 60.1±5.8. In acute VKH uveitic phase associated with exudative retinal detachment (n=12), arteriolar and venular oxygen saturation values were 104.7±7.8 and 67.9±7.7, respectively, and both are significantly higher than the healthy group (p<0.001; p=0.001, respectively). In patients with VKH disease who recovered after immunosuppressive therapy and restored normal anatomy without ‘sunset glow fundus’ (n=13), oximetry values were 96.4±9.6 and 61.6±7.5, respectively, similar to healthy controls. In patients with ‘sunset glow fundus’ and chorioretinal atrophy (n=16), saturation levels were 88.6±7.8 and 50.0±13.1, respectively, significantly lower than healthy controls (p=0.02; p=0.003, respectively). These patients also had significantly smaller diameter of retinal arterioles and venules compared with controls (p=0.035; p=0.001, respectively).ConclusionsRetinal oxygen metabolism is altered in uveitis associated with VKH disease. Oxygen saturation profile is abnormal in acute uveitic phase of the disease and returns to normal in those who recover with normal fundus appearance, but not in eyes that suffer permanent anatomical damage with ‘sunset glow fundus’ and chorioretinal atrophy. Retinal oximetry may be of value in evaluating vascular and metabolic aspects of posterior uveitis.

Pseudotumoral and Multiple Retinal Pigment Epithelium Proliferation in Vogt-Koyanagi-Harada Disease

We report a case of pseudotumoral retinal pigment epithelium (RPE) proliferation in Vogt-Koyanagi-Harada (VKH) disease, in a 50-year-old female who presented with a juxtapapillary and peripheral subretinal hyperpigmented lesions in the left eye and “sunset glow fundus,” hyperpigmented striae, and multiple atrophic chorioretinal spots in the periphery. The darkly pigmented exuberant larger subretinal mass extended to the periphery with associated subretinal fibrosis. This patient demonstrated the entire clinical presentation of VKH disease, which tends to course with a chronic, bilateral, granulomatous panuveitis and exudative retinal detachment associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. Our case is unique for the presence of exuberant, pseudotumoral RPE proliferation at the juxtapapillary region and peripheral area. Although this complication has rarely been reported, a high index of suspicion is warranted for early diagnosis and avoids unnecessary treatments of a pseudotumor.