Vogt-Koyanagi-Harada syndrome (VKH) is an autoimmune disorder primarily characterized by bilateral granulomatous uveitis, which can lead to severe visual impairment and related complications. Traditional treatment typically involves glucocorticoid combined with immunosuppressants, but these therapies are associated with significant side effects, limited efficacy, and poor long-term prognosis. In recent years, biologic agents have emerged as a promising treatment for refractory VKH due to their targeted action, high efficacy, and low toxicity. Tumor necrosis factor-alpha (TNF-α) inhibitors, such as infliximab and adalimumab, have shown significant benefits in controlling inflammation, improving vision, and reducing steroid dependence, making them a key option for difficult-to-treat VKH. Among interleukin (IL) blockers, tocilizumab has demonstrated potential in patients who do not respond to traditional treatments. Rituximab, a B-cell targeting agent, has shown good efficacy and safety in patients resistant to TNF-α inhibitors. Additionally, research into novel biologics targeting the IL-23/IL-17 axis and IL-33 offers new directions for VKH therapy. While biologics provide clear advantages in VKH treatment, further research is needed to explore their long-term safety, cost-effectiveness, and optimal treatment regimens. Large-scale randomized controlled trials are required to validate their efficacy and identify personalized treatment strategies to improve long-term patient outcomes.
Objective To investigate the imaging characteristics and clinical significance of rod-cone detachment (BALAD) in patients with acute VKH. MethodsA retrospective clinical study. A total of 125 patients (250 eyes) with acute VKH syndrome diagnosed by multimodal imaging in Department of Ophthalmology of Yunnan University Affiliated Hospital from January 2018 to December 2024 were included in this study. All affected eyes underwent fundus color photography, fluorescein fundus angiography (FFA), and optical coherence tomography (OCT) examinations. According to the OCT examination results, the affected eyes were divided into the BALAD combined group and the non-BALAD combined group. All patients received systemic oral prednisone acetate treatment. The multimodal imaging features of BALAD was observed and the clinical prognostic indicators of the two groups were compared at 6 months after treatment. The χ2 test was used for comparison between groups. ResultsAmong the 250 eyes, 168 eyes (67.2%) were in the combined BALAD group and 82 eyes (32.8%) were in the non-combined BALAD group. Fundus color photography examination showed BALAD as a circular or irregular bulge at the posterior pole. FFA examination showed weak fluorescence in the early stage of the lesion area, and fluorescein leakage occured in the late stage, but its fluorescence intensity was slightly weaker than that of the surrounding leakage area. OCT examination showed that BALAD presents a typical cyst-like space within the retina, with a continuous epiretinal membrane covering the top and a thickened ellipsoidal zone (EZ) at the bottom. Moreover, the EZ in this area remains continuous with those outside the BALAD region. The retinal pigment epithelial layer showed wavy changes. According to the morphology and density of the exudate in the cyst cavity, it can be classified into mild and micro-exudate type (a small amount of punctate strong reflex, 42 eyes), dense exudate type (relatively dense punctate exudate, 114 eyes), and fibrous exudate type (flocculent or cord-like exudate, 12 eyes). Six months after treatment, the EZ defect rate and the recurrence rate of VKH syndrome in the BALAD combined group were significantly higher than those in the non-BALAD combined group (χ2=0.547, 5.768; P < 0.05). In terms of prognosis, 42 eyes with mild and micro exudation responded well to the treatment. Among the 114 eyes with dense exudation, 70 eyes responded well. The treatment response was poor in 12 eyes with fibrous exudation type, among which 6 eyes eventually formed fibrous scars. ConclusionsBALAD is a common OCT image in patients with acute VKH, with a more typical fundus manifestation. The occurrence of BALAD is correlated with damage to the EZ band and recurrence of VKH.
The pathogenesis of Vogt-Koyanagi Harada disease (VKH) has not yet been fully defined. Current studies mainly suggest that VKH is actually an autoimmune disease, especially related to the immune response mediated by various signal transduction pathways involved in the function of T cells. In recent years, the influence of the balance imbalance of various T cell subsets in cellular immunity on the pathogenesis of VKH has been a hot research direction. Currently, T helper cell 17/T regulatory cells, balance is the focus of clinical research, meanwhile, new discoveries and potential clinical treatment schemes have been made for related cellular pathways, particularly the Janus kinase/signal transducers and activators of transcription pathway and NF-kappa B pathway. The exploration of B cells in the pathogenesis of VKH has also achieved initial results through the successful application of various targeted drugs. In the future, further screening and localization of genes or proteins that are abnormally regulated or expressed in VKH, for which early comprehensive and in-depth exploration will be helpful, thus improve the efficacy of clinical treatment programs and develop new therapeutic targets.
Objective
To investigate the cilinical value of indocyanine green angiography(ICGA) in patients with Vogt-Koyanagi-Harada syndrome(VKH).
Methods
Fundus fluorescein angiography(FFA) and indocyanine green angiography(ICGA) were used for comparative analyses in 26 cases(52 eyes)of VKH.
Results
In the acute stage of VKH,FFA revealed the multifocal leakage in the pigment epithelium and the multifocal serous retinal detachment,and the typical FFA manifestations disappeard following treatment．In the acute stage of the disease the ICGA showed:(1)numerous patchy areas of hypofluorescence and decreased flurescence in large and middle choroidal vessels(66.7%);(2)dilatation of the choroidal vessels(70.8%)and(3)in latephase of ICGA,the patchy areas of hyperfluorescence(79.2%).During the recovery stage of the disease,the abnormal undings in ICGA were resolved slower than those found in FFA.
Conclusions
ICGA may assist in providing valuable informations on choroidal circulation of VKH and be useful in evaluating the curative effects．
(Chin J Ocul Fundus Dis,20000,16:9-11)
