Bilateral peripapillary choroidal neovascular membranes in Vogt-Koyanagi-Harada disease.

Choroidal neovascular membrane (CNVM) in Vogt-Koyanagi-Harada disease (VKH) is a known entity, observed primarily during the chronic convalescent and chronic-recurrent phases of the disease. However, the peripapillary location of CNVM is a rare finding.We describe a case of chronic VKH with bilateral peripapillary CNVM detected using multimodal imaging and the associated differential diagnoses and treatment approach.A combination of anti-vascular endothelial growth factor injections, systemic steroids and immunosuppressants is often required to manage the aggressive course of this choroidal neovascularisation.

A case report: co-occurrence of probable Vogt-Koyanagi-Harada disease and diabetic retinopathy.

BACKGROUND: Bilateral retinal detachment and choroidal detachment in a patient are rare occurrences. The presence of bilateral diabetic retinopathy (DR) in such a case is even rarer and complicates the condition. CASE PRESENTATION: In this study, we document a case of unconventional VKH. Manifestations in this patient included intense peripheral retinal detachment and choroidal detachment, along with vitreous opacities akin to cotton wool spots, concurrent with DR. The diagnosis was considered as probable VKH with DR. Treatment according to VKH protocols, including high-dose corticosteroids, yielded positive results. CONCLUSIONS: VKH can co-occurrence with DR. VKH manifestations vary, and early, aggressive, and long-term treatment is essential. The complexity of treatment increases with concurrent DR, necessitating the use of immunosuppressants.

Surgical management of a case of non-resolving bullous retinal detachment due to probable Vogt-Koyanagi-Harada syndrome.

A 45-year-old male with Vogt-Koyanagi-Harada (VKH) syndrome presented with vision loss in his right eye after discontinuing treatment during the COVID-19 pandemic. He was found to have bullous retinal detachment (RD) in the right eye and was started on subcutaneous adalimumab with oral corticosteroid following three doses of pulse corticosteroid. But when RD did not resolve after 4 months of treatment and ultrasound B scan showed bullous RD with retino-retinal adhesion, he was planned for surgical intervention. During surgery, there was retino-retinal adhesions due to long-standing "kissing exudative RD", causing non-settling exudative RD. Following surgery, the vision improved to 2/60, with attached retina. The patient has been under follow-up with us for the last one year now and developed no recurrence of RD till now. This case emphasises the significance of retinoretinal adhesion in long-standing bullous RD that does not respond to conventional aggressive medical therapy.

VKH with APECED in a Two-Year-Old Child: A Rare Concomitant Diagnosis in an Unprecedented Age.

PURPOSE: To present a case of Vogt Koyanagi Harada (VKH) associated with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED) in a two-year-old. CASE PRESENTATION: A two-year-old type 1 diabetic with hypothyroidism presented with impaired fixation. Ocular examination revealed right vitritis, choroiditis, a hyperemic disc, and an area of exudative detachment. At the same time, there was no fundus view in the left eye, and ultrasonographic assessment revealed vitritis and a thickened choroid. Patient developed sunset glow fundus with alopecia, poliosis and vitiligo and a diagnosis of complete VKH with APECED was made. CONCLUSION: APECED is a rare endocrine disorder and has been reported to be associated with VKH twice. Likewise, VKH is commonly present in much older patients; this is the first time ever to be diagnosed in a two-year-old child.

Comparisons of choroidal blood flow velocity between initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease and acute central serous chorioretinopathy.

OBJECTIVES: Acute central serous chorioretinopathy (CSC) and Vogt-Koyanagi-Harada (VKH) disease in the acute uveitic phase are characterized by serous retinal detachment caused by dysfunction of the choroid. The aim of this study is to compare blood flow velocity and pulse waveform parameters in the choroid between these two diseases. METHODS: In this study, 25 patients (50 eyes) with VKH disease, 21 patients (27 eyes) with CSC and 15 healthy controls (30 eyes) were studied. Laser speckle flowgraphy (LSFG) was performed at presentation. RESULTS: Choroidal mean blur rate (MBR), representing blood flow velocity in choroidal vessels, was significantly lower in the eyes affected by VKH disease compared with the healthy control and CSC eyes. CSC eyes had a significantly higher MBR compared with healthy controls. Among the analyzed pulse waveform parameters, blow-out time (BOT), falling rate (FR) and flow acceleration index (FAI) changed significantly. BOT value was significantly lower in CSC eyes than in healthy control and VKH eyes. FR and FAI values were significantly lower in VKH eyes than in healthy control and CSC eyes. There was a strong positive correlation between MBR and FAI. CONCLUSIONS: Our findings confirm different pathophysiology of these two diseases. Assessment of choroidal blood flow velocity and haemodynamics with LSFG provides useful information to differentiate acute CSC and initial-onset acute uveitis associated with VKH disease.

Prognostic value of pretreatment indocyanine green angiography in the acute uveitic phase of Vogt-Koyanagi-Harada disease.

PURPOSE: To investigate the prognostic value of pretreatment indocyanine green angiographic (ICGA) features in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease. METHODS: Retrospective analysis of 84 patients (168 eyes). Main outcome measures were final visual acuity, development of 'sunset glow fundus' (SGF) and progression to chronic recurrent evolution. RESULTS: Thirty-eight patients (76 eyes) presented in the phase preceding anterior segment (AS) inflammation (early presentation) and 46 patients (92 eyes) had AS inflammation at presentation (late presentation). The mean number of hypofluorescent dark dots (HDDs) and frequency of disc hyperfluorescence were more in the late presentation group (p < 0.001 for both comparisons), whereas the early presentation group showed higher frequencies of peripapillary punctate choroidal hyperfluorescence (p < 0.001) and hypofluorescent patches involving macula corresponding to the areas of exudative retinal detachment (p = 0.012). The mean number of HDDs and the frequency of disc hyperfluorescence were higher among eyes that developed SGF (p < 0.001 for both comparisons) and eyes that progressed to chronic recurrent evolution (p < 0.001; p = 0.001, respectively). The frequencies of peripapillary punctate choroidal hyperfluorescence and hypofluorescent patches corresponding to the areas of exudative retinal detachment were less in the eyes that developed SGF (p = 0.019; p = 0.003, respectively). Punctate choroidal hyperfluorescence elsewhere was less frequent in the eyes that developed SGF (p < 0.001) and eyes that progressed to chronic recurrent evolution (p = 0.002). CONCLUSIONS: Pretreatment ICGA has a prognostic value in initial-onset acute uveitis associated with VKH disease.

Epidemiology of Pediatric Uveitis.

Uveitis is uncommon in children and its diagnosis and treatment are challenging. Little is known of the epidemiology of pediatric uveitis. Indeed, population-based studies in the literature are rare. However, there are many tertiary referral center reports that describe the patterns of uveitis in childhood, although few are from developed countries, and their comparison presents some issues. Anterior uveitis is the most frequent entity worldwide, especially in Western countries, where juvenile idiopathic arthritis is diffuse. Most cases of intermediate uveitis do not show any association with infectious or noninfectious systemic diseases. In low- and middle-income countries, posterior uveitis and panuveitis are prevalent due to the higher rates of infectious etiologies and systemic diseases such as Behçet disease and Vogt-Koyanagi-Harada disease. In recent decades, idiopathic uveitis rate has decreased thanks to diagnostic improvements.

Incidence and Risk Factors of Recurrent Anterior Uveitis in Initial Acute-Onset Vogt-Koyanagi-Harada Disease.

PURPOSE: We report the estimated incidence of, and risk factors for, recurrent anterior uveitis in patients with initial acute-onset Vogt-Koyanagi-Harada (VKH) disease using survival analyses. METHODS: Patients who were diagnosed with initial acute-onset VKH disease during 2003-2022 at two university hospitals were included. Recurrent anterior uveitis was defined as the first occurrence of the granulomatous anterior uveitis with anterior chamber cells and flare of 2+ or more by the Standardization of Uveitis Nomenclature (SUN) Working Group grading scheme, after the disappearance of conspicuous uveitis and serous retinal detachment for at least 3 months, regardless of systemic or local treatment. The univariate log-rank test and multivariate Cox regression analyses were performed, including patients' demographic characteristics, underlying diseases, presence of prodromal symptoms, duration of visual symptoms, visual acuity, slit-lamp and fundus findings, and height of serous retinal detachment. The treatment method and response to treatment were also included. RESULTS: The estimated incidence was 39.3% at 10 years. Fifteen of 55 patients (27.3%) had recurrent anterior uveitis during the mean follow-up of 4.5 years. The presence of focal posterior synechiae at the diagnosis increased the risk of recurrent anterior uveitis 6.97-fold compared to the absence of synechiae (95% confidence interval [CI], 2.20-22.11; p < 0.001). Use of systemic high-dose steroid therapy more than 7 days after the development of visual symptoms resulted in a hazard ratio of 4.55 (95% CI, 1.27-16.40; p = 0.020). CONCLUSIONS: This study reports the estimated incidence and risk factors of recurrent anterior uveitis in VKH disease from survival analyses. However, because of the retrospective nature of this study, it is hard to confirm the consistency of the medical records regarding risk factors, thus, the presence of focal posterior synechiae can be inconclusive as a risk factor. Further studies are warranted.

A COVID-19 perspective of Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada (VKH) disease, a bilateral granulomatous panuveitis associated with multisystem involvement, is a T-cell-mediated autoimmune disorder in which cytotoxic T-cell target melanocytes in genetically susceptible individuals. Recently, there has been an increase in literature on the new onset of uveitis and reactivation of previously diagnosed cases of uveitis following Covid-19 vaccinations. It has been postulated that Covid-19 vaccines can lead to an immunomodulatory change resulting in an autoimmune phenomenon in the recipients. VKH following COVID-19 infection was reported in four patients and a total of 46 patients developing VKH or VKH-like disease following COVID-19 vaccinations. There are reports of four patients who had been recovering or recovered from VKH after receiving the first dosage of the vaccine and developed worsening of ocular inflammation after receiving the second dose of the vaccine.

Clinical features, diagnosis, and management of COVID-19 vaccine-associated Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada (VKH) disease is a rare and serious ocular adverse reaction following COVID-19 vaccination. This study aimed to evaluate the clinical features, diagnosis and management of COVID-19 vaccine-associated VKH disease. Case reports of VKH disease after COVID-19 vaccination were collected up to February 11, 2023 for retrospective analysis. Twenty-one patients (9 males and 12 females) were included, with a median age of 45 years (range 19-78), from three main regions, Asia (12/21), the Mediterranean region (4/21), and South America (5/21). Fourteen patients developed symptoms after the first dose of the vaccine, and 8 after the second dose. Vaccines included mRNA vaccine (10 cases), virus vector vaccine (6 cases), and inactivated vaccine (5 cases). The average time interval from vaccination to onset of symptoms was 7.5 days (range 12 hours to 4 weeks). All 21 patients experienced visual impairment after vaccination, with 20 cases involving both eyes. Sixteen patients showed symptoms of meningitis. Serous retinal detachment was observed in 16 patients, choroidal thickening was observed in 14, aqueous cell in 9, and subretinal fluid in 6. CSF pleocytosis was detected in 7 patients and skin symptoms were found in 3 patients. All patients received corticosteroid therapy, and 8 also received immunosuppressive agents. All patients recovered well, with a mean recovery time of 2 months. Early diagnosis and early treatment are crucial to the prognosis of patients with VKH after vaccination with COVID-19 vaccine. The risk of vaccination against COVID-19 in patients with a history of VKH disease should be evaluated clinically.

CLINICAL FEATURES AND PROGNOSTIC VALUE OF BACILLARY LAYER DETACHMENT IN ACUTE VOGT-KOYANAGI-HARADA DISEASE.

PURPOSE: To evaluate the characteristics of bacillary layer detachment (BALAD) in acute Vogt-Koyanagi-Harada (VKH) disease and determine its prognostic value. METHODS: Seventy patients with acute VKH disease with a minimum follow-up of 6 months were studied. The primary outcomes were clinical characteristics associated with BALAD, including features on multimodal imaging at baseline and follow-up. The secondary outcomes included best-corrected visual acuity and VKH with recurrence features. RESULTS: Of 70 eyes (36 patients), 41 (58.6%) showed BALAD. The mean baseline best-corrected visual acuity and mean best-corrected visual acuity after resolution of serous retinal detachment were significantly lower in the BALAD group than in the no-BALAD group (0.90 ± 0.49 vs. 0.35 ± 0.35 log minimum angle of resolution, P < 0.001 and 0.39 ± 0.27 vs. 0.20 ± 0.20 log minimum angle of resolution, P = 0.020). The loss of ellipsoid zone integrity at baseline, proportion of serous retinal detachment, duration of serous retinal detachment, loss of ellipsoid zone integrity at 1 month, and subfoveal choroidal thickness at baseline were significantly higher in the BALAD group ( P = 0.017, P = 0.006, P = 0.023, P = 0.002, and P = 0.046, respectively). The mean best-corrected visual acuity and subfoveal choroidal thickness did not differ between the two groups at 6 months ( P = 0.380 and P = 0.180, respectively). Bacillary layer detachment at baseline was found to be a significant prognostic factor for VKH with recurrence features ( P = 0.007). CONCLUSION: Vogt-Koyanagi-Harada with BALAD featured more severe clinical characteristics than VKH without BALAD during the acute phase. Patients with baseline BALAD require more vigilant monitoring as they are more likely to show recurrence features within the first 6 months.

Comprehensive profiling of extracellular vesicles in uveitis and scleritis enables biomarker discovery and mechanism exploration.

BACKGROUND: Uveitis and posterior scleritis are sight-threatening diseases with undefined pathogenesis and accurate diagnosis remains challenging. METHODS: Two plasma-derived extracellular vesicle (EV) subpopulations, small and large EVs, obtained from patients with ankylosing spondylitis-related uveitis, Behcet's disease uveitis, Vogt-Koyanagi-Harada syndrome, and posterior scleritis were subjected to proteomics analysis alongside plasma using SWATH-MS. A comprehensive bioinformatics analysis was performed on the proteomic profiles of sEVs, lEVs, and plasma. Candidate biomarkers were validated in a new cohort using ELISA. Pearson correlation analysis was performed to analyze the relationship between clinical parameters and proteomic data. Connectivity map database was used to predict therapeutic agents. RESULTS: In total, 3,668 proteins were identified and over 3000 proteins were quantified from 278 samples. When comparing diseased group to healthy control, the proteomic profiles of the two EV subgroups were more correlated with disease than plasma. Comprehensive bioinformatics analysis highlighted potential pathogenic mechanisms for these diseases. Potential biomarker panels for four diseases were identified and validated. We found a negative correlation between plasma endothelin-converting enzyme 1 level and mean retinal thickness. Potential therapeutic drugs were proposed, and their targets were identified. CONCLUSIONS: This study provides a proteomic landscape of plasma and EVs involved in ankylosing spondylitis-related uveitis, Behcet's disease uveitis, Vogt-Koyanagi-Harada syndrome, and posterior scleritis, offers insights into disease pathogenesis, identifies valuable biomarker candidates, and proposes promising therapeutic agents.

COVID-19 Developed During Systemic Steroid Therapy for Vogt-Koyanagi-Harada Disease: A Case Report.

PURPOSE: The use of immunomodulatory therapy in the setting of coexistence of uveitis and coronavirus disease (COVID-19) remains controversial. We report a case of COVID-19 during systemic steroid therapy for Vogt-Koyanagi-Harada disease (VKH). CASE REPORT: A 43-year-old female was diagnosed with VKH and started on steroid pulse therapy (1,000 mg/day) followed by high-dose oral corticosteroids. Two weeks after discharge from the hospital, she was readmitted to the intensive care unit with severe acute respiratory syndrome due to SARS-CoV-2 infection confirmed by PCR test, and fortunately both VKH and COVID-19-induced respiratory disease improved. CONCLUSION: Given the absence of international agreement on how to manage COVID-19 patients with steroid-dependent VKH, existing clinical guidelines should be reviewed thoroughly to formulate useful strategies for managing VKH patients on steroid treatment who contract COVID-19. Furthermore, the outcomes of patients with steroid-dependent autoimmune uveitis including VKH who develop COVID-19 should be analyzed.

Sympathetic ophthalmia in an 85-year-old female and a 90-year-old male after a non-complicated cataract surgery: a case report.

Here, two cases involving the oldest reported patients with sympathetic ophthalmia (SO) after non-complicated cataract surgery, are reported. The first case was an 85-year-old female with bilateral decreased vision and headache. The best-corrected visual acuity (BCVA) was 0.8 in the right eye and 1.0 in the left eye at the initial visit. Pseudophakia and choroidal detachments were observed in both eyes, and retinal pigment epithelium undulation was observed by optical coherence tomography. The second case was a 90-year-old male with bilateral optic disc oedema. The BCVA was 0.09 in the right eye and 0.3 in the left eye with pseudophakic eyes. Optic disc oedema and choroidal thickening were observed in both eyes. Both patients were diagnosed with SO, and corticosteroid pulse treatments were started. The BCVA of the first case improved to 0.9 in the right eye and 1.2 in the left eye, while that of the second case reached 0.3 in the right eye and 0.6 in the left eye. No recurrence was observed in either case at 12 months after the initial visit. Both elderly patients with SO were successfully treated.

Incidence and pre/post-treatment risk factors of glaucoma in Vogt-Koyanagi-Harada disease.

PURPOSE: To investigate the incidence and pre/post-treatment risk factors of glaucoma in patients with Vogt-Koyanagi-Harada (VKH) disease. METHODS: Data regarding secondary glaucoma were collected from the medical records of patients with VKH disease who were followed up at the uveitis service at Hiroshima University for more than 6 months. We examined the incidence of glaucoma and pre/post-treatment risk factors for glaucoma in patients with VKH disease. RESULTS: Forty-nine patients with VKH disease were included in this study (31 women and 18 men). The mean age at onset was 50.4 ± 15.4 years and the mean length of follow-up was 40.7 ± 25.5 months. The most common initial treatment was pulse intravenous corticosteroid therapy (89.8%). Fifteen patients developed secondary glaucoma during follow-up. The median time of glaucoma onset from VKH development was 4.5 months (range 0-44 months). Disc swelling type as a pre-treatment factor (p = 0.089, hazard ratio = 7.268), worse final best corrected visual acuity (p = 0.099, odds ratio = 1.545), and cataract progression (p = 0.076, odds ratio = 7.886) as post-treatment factors showed trends for glaucoma development. The patients who progressed to the chronic recurrent stage had more complications including glaucoma. CONCLUSION: Secondary glaucoma occurred in more than 30% of patients with VKH disease. The factors that showed a trend toward glaucoma development may reflect an association with delayed treatment initiation and prolonged ocular inflammation.

Successive onset of Vogt-Koyanagi-Harada syndrome in father and son.

BACKGROUND: Vogt‒Koyanagi‒Harada (VKH) disease is a multifactorial systemic autoimmune disorder against melanocytes that is characterized by panuveitis. Familial occurrence of VKH disease is rare. Here, we report two cases of a father and his son with characteristic manifestations of VKH disease. CASE PRESENTATION: A 53-year-old male with typical clinical symptoms of VKH disease was referred to Tangshan Eye Hospital. Examination showed the presence of ciliochoroidal effusion and exudative retinal detachment in both eyes. The patient was given intravenous methylprednisolone 120 mg for 2 days and intravenous methylprednisolone 80 mg for 1 day followed by 48 mg (1 mg/kg/day) oral methylprednisolone daily, accompanied by oral azathioprine 50 mg daily. Cycloplegic agent (0.5% tropicamide three times daily [TID]) was added. The patient was free of symptoms and recurrence within more than 1-year-follow-up period, the best corrected visual acuity (BVCA) was increased and maintained in both eyes with complete resolution of subretinal fluid. One year and nine months later, case 2 (his son) also presented with the typical clinical symptoms of VKH disease at 29 years of age. The son also recovered from VKH disease after routine and standard treatment. CONCLUSIONS: To the best of our knowledge, this is the first VKH disease case report of a father-son relationship. Although genetic factors have been demonstrated to be involved in the pathogenesis of VKH disease, the different inheritance modes of VKH patients need to be further explored and studied.

Ocular manifestations of vitiligo: a systematic review.

Vitiligo is a disorder characterized by loss of epidermal melanocytes, resulting in depigmented macules and patches. While the relationship between ocular pathology and vitiligo has been demonstrated in conditions such as Vogt-Koyanagi-Harada and Alezzandrini syndromes, the ocular associations of non-syndromic vitiligo are incompletely understood. We conducted a systematic review to comprehensively describe the structural and functional changes seen in the eyes of patients with vitiligo, to identify patients at heightened risk for ocular disease, and to provide an approach to management of ocular manifestations of vitiligo. Overall, the strongest link between vitiligo and ocular pathology seems to lie with dry eye disease and pigmentary abnormalities of various ocular structures, especially the retinal pigment epithelium. Normal-tension glaucoma may also be more prevalent in the vitiligo population. The available literature did not provide conclusive evidence for increased risk of cataracts or uveitis. Aside from the impact of symptomatic dry eye disease, it seems unlikely that there are significant functional consequences of these ocular manifestations such as impaired visual acuity or visual fields.

Pattern of Uveitis at a Tertiary Eye Hospital in Eastern Province of Saudi Arabia and Comparison with Local Literature.

PURPOSE: To explore the patterns of uveitis in a main ophthalmic referral center in the Eastern province of Saudi Arabia. METHODS: A retrospective chart review of uveitis cases was conducted in a tertiary eye hospital for five years. In addition, a comprehensive review of Saudi publications about uveitis patterns in the literature. RESULTS: Out of 222 patients included, 54.4% were females, with a mean age of 38.5 years. Uveitis was bilateral in 55.7%, non-granulomatous in 55.9%, and infectious in 11.3%. Most of the cases were anterior uveitis (51.1%), followed by panuveitis (26%), intermediate uveitis (12.8%), and posterior uveitis (10%). Apart from idiopathic cases (43.7%), the most common diagnoses were Vogt-Koyanagi-Harada disease (11.3%) and Behcet's disease (7.7%) in adults. Juvenile idiopathic arthritis (7.2%) was commonest among children. Posterior synechiae was the leading complication (32.0%), followed by cataracts (22.8%). CONCLUSIONS: VKH and Behçet disease were the primary non-idiopathic etiologies in Saudi Arabia.