Vogt-Koyanagi-Harada disease: the step-by-step approach to a better understanding of clinicopathology, immunopathology, diagnosis, and management: a brief review

Abstract

Background Appraisals of Vogt-Koyanagi-Harada disease (VKH) have become progressively more complete, since its first description in 1906. The availability of new investigational methods has improved our knowledge of the immunopathology, clinicopathology, diagnosis, and management of VKH disease. This review aimed to describe some of the steps that led to better characterization of VKH as a clinical entity. Methods We searched on PubMed for articles that described the history of VKH disease and analyzed the progress in disease appraisal with new investigational and imaging methods. In particular, we searched for articles that investigated the clinicopathology, diagnosis, and management of VKH. Findings The following developments were considered essential for improving the appraisal and understanding of VKH: (1) the history of the disease, (2) immunopathological mechanisms, (3) clinicopathology, (4) the importance of distinguishing initial-onset from chronic disease, (5) relevant imaging modalities, among which indocyanine green angiography is crucial, (6) diagnostic criteria that facilitate early diagnosis, and (7) the need for early, prolonged, aggressive treatment that combines steroidal and non-steroidal immunosuppression. Conclusion Based on these findings, the definition of VKH has improved. VKH disease starts in the choroidal stroma and later involves other structures when it is not diagnosed and treated early. Indocyanine green angiography and enhanced depth imaging optical coherence tomography facilitate early diagnosis and precise monitoring of choroidal inflammation. ICGA is clearly the gold standard for appraisals and follow-ups in VKH disease, however EDI-OCT should be especially considered in those areas where ICGA is not fully available. These modalities have contributed substantially to a “cure” for VKH, when treatment is introduced within the therapeutic window of opportunity.

Clinical relevance of fibrin membranous structures in the intra‐photoreceptor outer segment separation on spectral domain optical coherence tomography (SD‐OCT) in initial‐onset acute Vogt‐Koyanagi‐Harada disease

“…On the contrary, the severity of VKH disease without FMS was probably underestimated and undertreated, which explains the higher recurrence rate and higher proportion of SGF at 6 months. This confirms that initial‐onset VKH should be treated vigorously regardless whether FMS are present or not, as has been stressed repeatedly in the recent literature (Abu El‐Asrar et al, 2021; Herbort Jr. et al, 2017; Herbort Jr. et al, 2021; Papasavvas et al, 2020; Urzua et al, 2022).…”

“…VKH patients without FMS were older, more likely to be female, and having longer duration from the onset of symptoms to the first presentation. In the clinical classification that divides initial‐onset acute VKH disease into the SRD type and the optic disc (OD) swelling type, it was documented that OD swelling‐type VKH patients were older, relatively more female and had a longer interval between onset of symptoms and treatment initiation compared with SRD‐type VKH patients (Baltmr et al, 2016; Nakao et al, 2012; Okunuki et al, 2015; Urzua et al, 2022). These clinical features are corresponding to VKH patients without FMS.…”

“…The findings that VKH patients without FMS were older and more likely to be female might possibly result from age‐related changes in the immune system and/or from effects of sex hormones. However, duration from the onset of symptoms to first presentation was significantly longer in the non‐FMS group when compared to the FMS group, suggesting that the FMS and non‐FMS groups are not clinically different types but seem to represent different evolutionary time points in the clinical course of initial‐onset acute VKH disease, an explanation that may also apply to the sub‐division of the SRD type and the OD swelling‐type (Baltmr et al, 2016; Nakao et al, 2012; Okunuki et al, 2015; Urzua et al, 2022). We speculate that hyperfluorescence of the optic disc from the early stage on FA and attenuated HDDs on ICGA in VKH patients without FMS represent the natural course of initial‐onset acute self‐limited VKH disease.…”

“…Exclusion criteria were presence of corneal disease, primary glaucoma, exfoliation syndrome, history of trauma or vitrectomy, history of other uveitis, other systemic inflammatory disease or malignancy. Patients were also excluded if they presented chronicity criteria as described by Urzua et al (2022).…”

Adalimumab treatment for chronic recurrent Vogt-Koyanagi-Harada disease with sunset glow fundus

“…The study protocol was described to all human subjects by opt-out method, and written informed consent was waived by the ERB due to the retrospective nature of the study. The criteria and classification of VKH disease by the previous reports[ 28 29 ] were used to diagnose VKH patients. Exclusion criteria were the presence of corneal diseases, primary glaucoma, exfoliation syndrome, history of trauma or vitrectomy, history of other uveitis, other systemic inflammatory diseases, or malignancy.…”