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.

Classification of Non-Infectious and/or Immune Mediated Cho-Roiditis: A Brief Overview of the Essentials

The choroid was poorly accessible to imaging investigation until the last decade of the last century. With the availability of more precise imaging methods such as indocyanine green angiography (ICGA) and, later, optical coherence tomography (OCT), enhanced depth OCT (EDI-OCT), and OCT angiography (OCTA), appraisal of choroidal inflammation has substantially gained in accuracy. This allowed to precisely determine which structures were touched in the different non-infectious choroiditis entities and made it possible to classify this group of diseases, ICGA signs, mainly hypofluorescent lesions, were identified and described. Previous publications have divided angiographic findings into two main sets of signs: (1) irregular “geographic” hypofluorescent areas corresponding to choriocapillaris non-perfusion and (2) round more regular, hypofluorescent dark dots more evenly distributed in the fundus corresponding to more deep choroidal stromal foci. These distinct findings allowed to subdivide and classify choroiditis into choriocapillaritis and stromal choroiditis. Additional signs were identified from EDI-OCT and OCTA examination supporting the classification of choroiditis into choriocapillaritis and stromal choroiditis. Results: Diseases involving principally the choriocapillaris included Multiple Evanescent White Dot Syndrome (MEWDS), Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE), Idiopathic Multifocal Choroiditis (MFC), and Serpiginous Choroiditis (SC) as well as mixed forms. Diseases primarily involving the choroidal stroma included HLA-A29 Birdshot Retinochoroiditis (BRC), Vogt-Koyanagi-Harada disease (VKH), Sympathetic Ophthalmia (SO), and Sarcoidosis chorioretinitis (SARC). Thanks to new imaging investigations of the choroid, it is now possible to classify and understand the diverse clinicopathological mechanisms in the group of non-infectious choroiditis entities.

Vogt-Koyanagi-Harada disease is always bilateral: reports of unilateral cases failed to use choroidal investigations showing subclinical involvement of the fellow eye

Background/purpose Vogt-Koyanagi-Harada (VKH) disease is a primary stromal choroiditis and a bilateral granulomatous panuveitis which, if not treated early and properly, could have a deleterious evolution. The purpose of our case report is to demonstrate that “so called” unilateral VKH disease should be investigated further with an Indocyanine Green Angiography (ICGA), in order to detect subclinical choroidal involvement of the other eye. Case report We present a case of 42-year old woman who came to see us for second opinion. She had consulted elsewhere for a left uveitis and had been treated with a periocular corticosteroid injection. At presentation she mentioned persistent headaches. Visual acuity on the Snellen scale was 1.0 OD and 0.5 OS. Slit-lamp examination showed granulomatous (rare mutton-fat KPs) signs in her left eye. Laser flare photometry showed a subclinical flare of 17.8 ph/ms OD and a flare of 66.4 ph/ms OS (normal values 3–6 ph/ms). Fundus examination showed left discoloration due to choroidal infiltration with a normal fundus aspect OD. ICGA showed a diffuse choroiditis also in the apparently normal right eye. Lumbar puncture confirmed the diagnosis of VKH and appropriate treatment was introduced. Conclusion VKH disease results from a generalized autoimmune process against melanocyte associated antigens starting in the choroidal stroma. It can be asymmetrical but is always bilateral, as long as investigations such as ICGA, able to detect subclinical choroiditis, are performed.

Vogt-Koyanagi-Harada Disease and Birdshot Retinochoroidopathy, Similarities and Differences: A Glimpse into the Clinicopathology of Stromal Choroiditis, a Perspective and a Review

The purpose of this work was to give a comprehensive and updated review on two primary stromal choroiditis entities, Vogt-Koyanagi-Harada disease (VKH) and birdshot retinochoroiditis (BRC). Their appraisal has become much more precise thanks to new investigational methods, such as indocyanine green angiography (ICGA) and enhanced depth imaging optical coherence tomography (EDI-OCT), which give substantially improved imaging access to the choroid. In this review, we focus on the crucial changes brought by this progress in the understanding, diagnosis, and management of these disorders. Application of these methods makes it possible to reach an early diagnosis, therefore allowing early treatment, which has led to a profound improvement in outcomes when compared to previous management.

Need for Quantitative Measurement Methods for Posterior Uveitis: Comparison of Dual FA/ICGA Angiography, EDI-OCT Choroidal Thickness and SUN Vitreous Haze Evaluation in Stromal Choroiditis

Background/Purpose Quantitative methods for posterior uveitis are necessary for precise appraisal and follow-up of inflammation in practice and in clinical trials. The aim of this study was to assess fluorescein angiography (FA), indocanine green angiography (ICGA), and enhanced depth imaging optical coherence tomography choroidal thickness (EDI-OCT CT) in two stromal choroiditis entities, birdshot retinochoroiditis (BRC), and Vogt-Koyanagi-Harada disease (VKH), as well as to determine (1) disease patterns, (2) respective response to therapy, and (3) their potential utility in clinical trials in comparison to vitreous haze, the present standard outcome used in clinical trials. Methods This retrospective study included newly diagnosed patients with BRC and VKH, seen at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland. Angiographic signs were quantified using an established dual FA/ICGA scoring system for uveitis at presentation and on follow-up. FA/ICGA score ratios were compared between diseases to determine disease patterns. EDI-OCT CT was determined using a spectral domain instrument. Vitreous haze was determined using the SUN (Standardization of Uveitis Nomenclature) method. Results Among 1872 uveitis patients seen from 1995 to 2016, 8 newly diagnosed BRC patients (16 eyes) and 6 newly diagnosed VKH patients (12 eyes) had sufficient data for study inclusion. Patients with BRC and VKH at initial onset had mean FA scores of 16.1 ± 7.0 vs. 4.6 ± 2.1 (p < 0.0001), respectively, while mean ICGA scores were similarly high in the two diseases, 18.9 ± 3.6 (BRC) vs. 20.8 ± 7.5 (VKH). After therapy, FA and ICGA scores decreased significantly for both entities (− 60% of FA score and 55% of ICGA score in BRC vs. − 72% of FA score and − 87% for ICGA score in VKH). EDI-OCT CT decreased significantly in the two entities. Vitreous haze was almost absent in VKH and low in BRC. Conclusion Dual FA/ICGA scoring showed the diverse disease patterns of BRC and VKH; both the retina and choroid were involved at onset in BRC, whereas VKH was a pure choroidal disease with later spillover into the retina. Dual FA/ICGA allowed for the precise measurement of inflammation at onset and upon follow-up. EDI-OCT CT responded to therapy in both diseases but was found to be of limited use in this early/subacute disease phase because it lacked sensitivity to detect subclinical recurrences and was therefore only useful for long-term follow-up. Vitreous haze was low in both entities and thus useless as an inflammatory parameter.