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.

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.

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.

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.

Outcomes of initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease occurred during pregnancy.

PURPOSE: To investigate the outcomes of initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease that occurred during pregnancy. METHODS: This is a retrospective case series. RESULTS: During the period between January 2001 and December 2021, we identified 112 patients with initial-onset acute uveitis associated with VKH disease, 67 (59.8%) were females. Among the female patients, 10 (14.9%) patients (20 eyes) were pregnant. Of these patients, 5 patients presented in the first trimester, 3 in the second trimester and 2 in the third trimester. The follow-up period ranged from 8 to 108 months (mean 35.2 ± 28.3 months). At presentation, 8 (80%) patients had initial-onset acute VKH disease with anterior segment (AS) inflammation and 2 (20%) initial-onset acute VKH disease without AS inflammation. All patients were initially treated with systemic corticosteroids combined with cyclosporine. During follow-up period, none of the patients with initial-onset acute VKH disease without AS inflammation developed any complications. Complications including "sunset glow fundus" in 8 (40%) eyes, cataract in 2 (10%) eyes and subretinal fibrosis in 1 (5%) eye were recorded in patients with initial-onset acute VKH disease with AS inflammation. Four (40%) patients developed pregnancy-related complications, including abortion in 1 patient, systemic hypertension in 1 patient and premature rupture of membrane in 2 patients. There were no documented congenital anomalies in all born babies. Best-corrected visual acuity of ≥ 20/20 was achieved in 16 (80%) eyes at the final follow-up. CONCLUSION: Primary treatment with combined systemic corticosteroids and cyclosporine in initial-onset acute uveitis associated with VKH disease was safe and effective.

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.

Posterior ocular manifestations following BNT162b2 mRNA COVID-19 vaccine: a case series.

PURPOSE: To report the occurrence of posterior ocular adverse events following the administration of the BNT162b2 mRNA vaccine against SARS-CoV-2. METHODS: A retrospective consecutive case series, in which the medical files of patients presenting with ocular adverse events within 30 days of the vaccine inoculation, were analyzed. RESULTS: Four patients (2 females) were included in the study. The diagnoses included: posterior scleritis, paracentral acute middle maculopathy, herpes panuveitis, and Vogt-Koyanagi-Harada (VKH)-like uveitis. Three of the patients had no relevant ocular history, but the patient who developed scleritis was in remission without medical therapy for four years, until the flare-up, which occurred one day after the vaccine. All patients improved with treatment. CONCLUSION: Though a causal relationship cannot be definitively established, the temporal relationship suggests a possible link between the COVID-19 vaccine and the posterior ocular complications. The benefits of vaccination clearly outweigh the potential adverse effects; however, ophthalmologists should be aware of the potential for vaccine-associated uveitis.

Unusual neurologic manifestations of Vogt-Koyanagi-Harada disease: a systematic literature review.

BACKGROUND AND PURPOSE: The usual neurologic manifestations of Vogt-Koyanagi-Harada (VKH) disease include aseptic meningitis and headaches. We performed the present study to review all unusual neurologic manifestations reported in VKH disease to summarize them. METHODS: A literature search was performed in the English language on Scopus and Medline via PubMed from 1946 to July 31, 2021, by using the following terms: "Vogt Koyanagi Harada disease" OR "VKH disease" AND "Brain" OR "Spinal cord" OR "CNS" OR "Central nervous system" OR "Neurologic" OR "Peripheral nervous system" OR "Polyneuropathies. Our inclusion criteria were unusual neurologic manifestations of VKH disease. RESULTS: Our literature search yielded 417 total articles (PubMed = 334, Scopus = 83) from which 32 studies comprising 43 patients (22 men and 21 women, of which 62.8% were younger than 50 years) were included in this systematic literature review. Regarding the study design, all studies were case reports and published between 1981 and 2021. CNS involvement was the most reported (93%) in VKH disease. Peripheral nervous system involvement represents 7% of cases. The cerebral lesions were parenchymal inflammatory lesions in the white matter or posterior fossa with or no contrast enhancement (16.3%), leptomeningitis (9.3%), pachymeningitis (7%), meningoencephalitis (2.3%), ischemic stroke (4.6%), hemorrhagic stroke (2.3%), transient ischemic attack (2.3%), and hydrocephalus (2.3%). The optic nerve lesions were anterior ischemic optic neuropathy (20.9%) and optic neuritis (9.3%). Concerning spinal cord lesion, it was mainly myelitis (14%). CONCLUSION: This systematic literature review provides a summary of the different unusual neurologic manifestations reported in VKH disease.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY REVEALS PARADOXICALLY DECREASING CHOROIDAL THICKNESS AND INCREASING BLOOD FLOW IN REMITTING VOGT-KOYANAGI-HARADA SYNDROME.

PURPOSE: To assess changes in choroidal thickness and blood flow in active Vogt-Koyanagi-Harada syndrome and after remission using optical coherence tomography angiography. METHODS: This was a prospective study of patients with active early uveitis secondary to Vogt-Koyanagi-Harada syndrome. They underwent optical coherence tomography angiography imaging twice: at baseline and after remission on treatment. 3- × 3- and 6- × 6-mm choriocapillaris slabs were used to evaluate parafoveal adjusted flow index as a marker for choroidal blood flow. Mean choroidal thickness of 3 points (subfoveally and 2 points 300 µ m parafoveally) was also measured. RESULTS: Thirty-nine eyes of 25 patients were initially recruited. After excluding eyes with media opacity, submacular fibrosis, and choroidal neovascularization, 23 eyes of 14 patients were included. The mean follow-up period was 8.7 ± 2.5 months. Mean choroidal thickness in activity and remission was 581.65 ± 108.29 µ m and 318.34 ± 72.85 µ m respectively ( P < 0.01). Mean adjusted flow index in the 3- × 3-mm slabs activity and remission were 0.495 ± 0.027 and 0.519 ± 0.0336 ( P = 0.011), and the 6- × 6-mm slabs were 0.487 ± 0.037 and 0.517 ± 0.052 respectively ( P = 0.025). CONCLUSION: We demonstrate decreasing choroidal thickness with paradoxically increasing choroidal flow on optical coherence tomography angiography in remitting Vogt-Koyanagi-Harada syndrome. This may reflect inflammatory infiltrations or granulomas increasing choroidal thickness during activity and causing sluggish circulation of the choriocapillaris, and a reversal of this process with remission. These findings shed more light on the relationship between Vogt Koyanagi Harada syndrome and its underlying choroidal disturbances. Larger studies are needed to evaluate the efficacy of adjusted flow index in evaluating and predicting disease activity.

Outcomes of retinal pigment epithelial detachment in Vogt-Koyanagi-Harada disease: a longitudinal analysis.

BACKGROUND: The aim of this study was to report the clinical profile and outcomes of retinal pigment epithelial detachment (PED) in Vogt-Koyanagi-Harada (VKH) disease, and to evaluate the correlation between PED and the subsequent development of central serous chorioretinopathy (CSC) throughout the whole corticosteroid treatment course. METHODS: The retrospective study enrolled a total of 470 eyes with VKH, and 12 eyes with VKH and PED were recruited. Patients were divided into two groups according to the CSC onset or not throughout the whole course (the CSC group and non-CSC group). Best-corrected visual acuity (BCVA) improvement, and PED angle (PEDA, the angle between the two lines of the vertex of the lifted retinal pigment epithelium to the two edge points of the Bruch membrane) were compared between the two groups. RESULTS: CSC developed at the site of the PED in 5 of the 12 eyes with PED, while in the remaining 7 eyes PED gradually resolved following therapy. The prevalence of PED and CSC in VKH was 2.55% (12/470) and 1.06% (5/470), respectively. BCVA improvement in the non-CSC group was greater than that in the CSC group, but without a statistical difference (P = 0.25). PEDA was significantly smaller in the CSC group than in the non-CSC group (P = 0.03). CONCLUSION: PEDA is an ideal parameter to reflect hydrostatic pressure and stretches for RPE. As PED predisposes to the development of CSC in selected VKH eyes, PEDA may be a valuable predictive factor for the development of classic CSC in VKH cases.

Phacoemulsification after trabeculectomy in uveitis associated with Vogt-Koyanagi-Harada disease: intermediate-term visual outcome, IOP control and trabeculectomy survival.

PURPOSE: To evaluate the visual outcome, intraocular pressure control and trabeculectomy survival after phacoemulsification in eyes with prior trabeculectomy in uveitis associated with Vogt-Koyanagi-Harada disease (VKH). DESIGN: Retrospective comparative study. METHODS: Eyes with uveitic glaucoma associated with VKH who underwent mitomycin C (MMC)-enhanced trabeculectomy were included. Eyes were divided into two groups: the first study group included eyes that later underwent cataract surgery in the form of phacoemulsification, and the second control group included eyes that did not have cataract surgery. The main outcome measures were changes in the visual acuity, intraocular pressure (IOP), the number of antiglaucoma medications, IOP control and trabeculectomy survival. RESULTS: There were no significant differences in the final visual acuity (0.78 (±0.9) and 0.92 (±1.1), p = 0.80)) nor IOP (14.21 mmHg (±5.8) and 12.16 mmHg (±6.1), p = 0.29), but there was a difference in the antiglaucoma medications (1.58 (±1.5) and 0.53 (±1.0), p = 0.02) between the study and control group, respectively. There was no difference in the overall trabeculectomy survival (p = 0.381, Log Rank), but more eyes in the study group converted to qualified success after phacoemulsification and required more medications to control the IOP. CONCLUSION: Phacoemulsification after trabeculectomy seems to be a safe procedure in eyes with combined vision threatening complications of VKH, although the visual improvement was limited. Nevertheless, more medications were required to control the IOP, resulting in less absolute and more qualified trabeculectomy success. Therefore, patient counseling before surgery is essential.

Rheumatologic manifestations in a cohort of patients with Vogt-Koyanagi-Harada disease.

OBJECTIVES: Vogt-Koyanagi-Harada Disease (VKHD) is a systemic autoimmune disorder characterized by granulomatous panuveitis. Inflammatory rheumatic diseases (IRDs) are among the differential diagnosis of VKHD. However, current knowledge on the rheumatological aspects of VKHD is still limited. We aimed to investigate the prevalence of rheumatic conditions in VKHD patients. METHODS: VKHD patients were included in the study and they were reviewed in terms of the presence of any rheumatological manifestations. RESULTS: There were 18 patients with a female preponderance (83.3%, female). Inflammatory type of peripheral joint pain (11%) and sicca symptoms (33%) were the most common rheumatological findings. The frequency of spondyloarthritis-related features such as inflammatory back pain and HLA-B27 rate was not increased. None of the patients had radiographic sacroiliitis. Anti-nuclear antibody was positive in high titres nearly in 30% of the patients and three patients had antibodies against extractable nuclear antigens. Nailfold capillaroscopy was abnormal in about one-third of the patients. Pathergy test was negative in all cohorts. While angiotensin-converting enzyme was elevated in nearly 20% of the patients, there were no abnormalities on chest X-rays. CONCLUSION: VKHD shares some features with IRDs. The common features were mostly suggestive of connective tissue disease rather than SpA or rheumatoid arthritis.

Comparison of the clinical features between posterior scleritis with exudative retinal detachment and Vogt-Koyanagi-Harada disease.

PURPOSE: This study aims to analyse the differences in clinical characteristics between VKH disease and PS with exudative retinal detachment (ERD). METHODS: The medical records of 18 eyes of 12 patients with PS accompanied by ERD and 32 eyes of 16 patients with VKH disease were retrospectively reviewed. RESULTS: Single ERD was more common in PS, while hyperreflective dots, multiple ERD, retinal pigment epithelium folds were more common in VKH disease on OCT. Both posterior coat thickness and choroid thickness were higher in VKH eyes. "T" sign was observed in 6 of 18 eyes (33.3%) in the PS group, whereas in none of the eyes of VKH disease. No significant differences were shown in FA imaging between PS and VKH cases. Relapse occurred in 12 eyes (66.7%) in PS group, mainly in the posterior segment, while 6 eyes (18.8%) experienced recurrence in the anterior segment in VKH group. CONCLUSION: There are characteristic differences in multimodal imaging parameters and clinical course between VKH and PS with ERD.

High prevalence of angle-closure glaucoma in Vogt-Koyanagi-Harada disease.

PURPOSE: To determine the prevalence, clinical characteristics, and mechanisms of secondary glaucoma in Vogt-Koyanagi-Harada (VKH) disease. METHODS: This retrospective, longitudinal observational study analyzed the demographic data, disease stage, glaucoma development, intraocular pressure, best-corrected visual acuity, lens status, optic nerve, gonioscopy, management, and visual outcomes of VKH disease. Clinical features were used to categorize the stage of VKH disease. VKH eyes were divided into two groups, with or without glaucoma, undergoing further analysis, including statistical analysis. RESULTS: 305 eyes of 155 patients with VKH disease with a median follow-up of 22 months were included. Secondary glaucoma developed in 67 (22%) eyes, most of which (64.2%) had chronic recurrent VKH at presentation. Angle-closure was present in 55 (82.1%) of glaucoma eyes. Peripheral anterior and posterior synechiae were present in 58 (86.6%) and 51 (76.1%) eyes, respectively. Pupillary block and posterior synechiae resulted in iris bombé in 17 (25.4%) eyes with glaucoma. At the last visit, visual acuity was worse in eyes with glaucoma (p < 0.001). CONCLUSION: We found that angle-closure disease is a significant cause of secondary glaucoma in VKH. Eyes with glaucoma were more likely to present in the chronic recurrent stage of the disease.

Increased serum level of interleukin-33 in Vogt-Koyanagi-Harada correlates with disease activity.

OBJECTIVES: To measure the serum level of IL-33 in patients with Vogt-Koyanagi-Harada disease (VKH) and Behçet's uveitis (BU) in the Chinese Han population and investigate its associations with disease activity and clinical parameters. METHODS: Serum was collected from 41 VKH patients (16 active and 25 inactive patients), 60 BU patients (24 active and 36 inactive patients), and 36 healthy controls. The serum level of IL-33 was measured using the enzyme-linked immunosorbent assay (ELISA) method. Demographic features, clinical manifestations, and intraocular inflammation activity scores (anterior chamber cells score, anterior chamber flare score, and vitreal haze score) were recorded. RESULTS: The serum level of IL-33 significantly increased in all VKH patients, active VKH patients, and inactive VKH patients, as compared to healthy controls (p < 0.001, p < 0.001, and p = 0.002, respectively), and was higher in the active VKH than in the inactive VKH patients (p = 0.049). The serum level of IL-33 positively correlated with the anterior chamber cells score, vitreal haze score, and the annualized number of relapses in VKH patients (Rho = 0.359, p = 0.021; Rho = 0.344, p = 0.028; Rho = 0.537, p < 0.001, respectively). Serum IL-33 level was significantly associated with the annualized number of relapses in patients with BU (Rho = 0.361, p = 0.005). CONCLUSION: Serum IL-33 level is significantly increased in VKH patients in the Chinese Han population. IL-33 level is in positive correlation with the activity and relapses of VKH. Increased IL-33 might contribute to the pathogenesis of VKH and serve as a potential biomarker for VKH disease.

BACILLARY LAYER DETACHMENT IN ACUTE VOGT-KOYANAGI-HARADA DISEASE: A Novel Swept-Source Optical Coherence Tomography Analysis.

PURPOSE: To report the frequency, optical coherence tomography (OCT) findings, and visual and anatomic outcomes of patients with acute Vogt-Koyanagi-Harada disease presenting with the bacillary layer detachment (BLD) (intraretinal split at the photoreceptor inner segment myoid). METHODS: This was a retrospective analysis of a consecutive series of patients with Vogt-Koyanagi-Harada disease having a minimum follow-up of 6 months. All patients had swept-source OCT, fluorescein angiography, and indocyanine green angiography performed at baseline. The characteristics of serial swept-source OCT were recorded and analyzed. RESULTS: Sixty-two subjects (42 women; age: 34.2 ± 12 years) with Vogt-Koyanagi-Harada disease were included. 118 eyes (95.2%) had serous retinal detachment at presentation. 112 eyes (94.9%) showed the BLD at baseline. In 8 of 112 (7.1%) eyes with the BLD, the external limiting membrane at the anterior aspect of the BLD showed focal discontinuity. The interdigitation zone at the base of the BLD showed discontinuity in 53 of 112 (47.3%) eyes with the BLD. The ellipsoid zone could not be identified as a separate hyperreflective line at the base of the BLD in 102 of 112 eyes (91.1%). Bacillary layer detachments resolved within 3.4 ± 1.3 days after intravenous methylprednisolone therapy with improvement in the best-corrected visual acuity from 0.96 to 0.4 logarithm of the minimum angle of resolution (20/184 Snellen's equivalent) (P < 0.001). Resolution of serous retinal detachment was observed after 5.9 ± 2.6 days. CONCLUSION: In eyes with acute Vogt-Koyanagi-Harada disease, the BLD is a common finding and represents a split in the photoreceptor layer at the inner segment myoid and can be differentiated from serous retinal detachment using swept-source OCT. In addition, resolution of the BLD and photoreceptor recovery can be evaluated using serial swept-source OCT.

Initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease presenting with unilateral exudative retinal detachment despite bilateral choroidal involvement.

PURPOSE: To investigate the frequency of initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease presenting with unilateral exudative retinal detachment. METHODS: A retrospective case series. RESULTS: During the period between January 1998 and December 2020, we identified 135 patients with initial-onset acute uveitis associated with VKH disease. Among them, 5 (3.7%) patients were referred to have unilateral uveitis due to the presence of exudative retinal detachment in only one eye. Optical coherence tomography confirmed the presence of unilateral exudative retinal detachment, however, indocyanine green angiography (ICGA) revealed characteristic findings of bilateral granulomatous choroidal inflammation typical for initial-onset acute uveitis associated with VKH disease. CONCLUSIONS: Patients with initial-onset acute uveitis associated with VKH disease can present with unilateral exudative retinal detachment. ICGA assessment of the choroid revealed the presence of subclinical involvement of the fellow eyes.

Proliferative retinopathy as a feature of Vogt Koyanagi Harada Disease: a report of two cases.

BACKGROUND: Proliferative retinopathy is an uncommon feature of Vogt Koyanagi Harada (VKH) disease which might indicate poor uveitis control in these patients. We aim to describe the clinical features and outcome of management of proliferative retinopathy in 2 patients with VKH. CASE PRESENTATION: 19 and 33 years old females with VKH presented with unilateral proliferative retinopathy. Both patients had neovascularization of the optic disc (NVDs) and one patient had neovascularizations elsewhere (NVEs) and preretinal hemorrhage. Both patients had exudative retinal detachments (ERD). Systemic steroids and immunomodulatory agents were successfully used to control inflammation and achieve regression. One patient developed fibrous tissue formation at the disc area as well as an epiretinal membrane formation, for which she had pars plana vitrectomy with membrane peeling. Both patients had controlled inflammation with stable vision. CONCLUSIONS: Proliferative retinopathy can present variably in VKH patients and indicates persistent inflammation which is incompletely controlled. Proper uveitis control is sufficient to achieve regression of retinal neovascularization.

Pigmented paravenous retinochoroidal atrophy associated with Vogt-Koyanagi-Harada disease: a case report.

BACKGROUND: To describe a unique case of pigmented paravenous retinochoroidal atrophy that developed several years after Vogt-Koyanagi-Harada disease. CASE PRESENTATION: A 28-year-old woman presented with gradual vision loss in both eyes and nyctalopia for 2 years. Past medical history was relevant for Vogt-Koyanagi-Harada disease since the age of 19 and positive HLA-DR4. Funduscopic examination revealed perivascular pigmentary clumping and atrophic changes radiating from the optic disks. Spectral domain optical coherence tomography through the macula demonstrated perifoveal outer retinal layers loss with cystic degeneration. Fundus autofluorescence showed zonal areas of hypoautofluorescence corresponding to the areas of atrophy. Full-field electroretinogram identified mildly reduced scotopic and photopic responses. The patient was diagnosed with pigmented paravenous retinochoroidal atrophy. CONCLUSIONS: Pigmented paravenous retinochoroidal atrophy may be acquired after Vogt-Koyanagi-Harada disease. Pathogenesis of pigmented paravenous retinochoroidal atrophy may involve inflammatory-related precursors on a background of genetic predisposition.