Acute Vogt-Koyanagi-Harada Disease Research Articles

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

To evaluate the characteristics of bacillary layer detachment (BALAD) in acute Vogt-Koyanagi-Harada (VKH) disease and determine its prognostic value. 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. 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). 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.

The Role of Widefield Optical Coherence Tomography Angiography in the Diagnosis and Management of Acute Vogt–Koyanagi–Harada Disease

ABSTRACT Purpose We explore the choroid vasculature changes of acute Vogt–Koyanagi–Harada (VKH) disease using widefield optical coherence tomography angiography (OCTA). Methods In this retrospective, observational, longitudinal study, 16 patients with acute VKH disease (32 eyes, mean age: 42.19 ±13.66 years) were measured using widefield OCTA. Results In this study, we first described the multiple dark foci in choriocapillaris and Sattler’s layer in the panoramic montage of the five 12x12mm images in 30 eyes (93.8%) of acute VKH disease. OCTA follow-up in these 30 eyes demonstrated the diminished size and number of these dark foci in choriocapillaris and Sattler’s layer after the initiation of treatment. Conclusions Widefield OCTA enables noninvasive identification of characteristics of flow void at the level of superficial choroidal vessels in the acute phase and may be a novel valuable tool for diagnosis and monitoring of disease progression in VKH disease in the future.

Association of Retinal Pigment Epithelium Reflectivity on Optical Coherence Tomography with Recurrence of Vogt-Koyanagi-Harada Disease: A Retrospective Observational Study.

Despite the necessity of optical coherence tomography (OCT) for diagnosis and longitudinal monitoring in patients with Vogt-Koyanagi-Harada (VKH) disease, no studies have identified useful OCT markers for predicting recurrence in these patients. Although the precise reason for this remains unclear, one possibility is that infiltration of inflammatory cells into the choroid attenuates the OCT signal, making it difficult to precisely assess the structure of the choroid. Therefore, this study aimed to investigate changes in retinal pigment epithelium (RPE) reflectivity immediately above the choroid in eyes with acute VKH disease, as well as the association between RPE reflectivity and VKH disease recurrence. This single-centered retrospective observational study included 20 treatment-naïve patients with acute VKH disease presenting with serous retinal detachment (SRD) in the posterior pole at the initial visit between October 2015 and January 2020, as well as 15 healthy control eyes. All patients were followed up for at least 6 months and received treatment with intravenous methylprednisolone followed by oral administration of prednisolone. Swept-source OCT images through the fovea were used to measure central retinal thickness, central choroidal thickness, and RPE reflectivity. During an observation period of 37.2 ± 30.8 months, recurrence of inflammation was observed in 11 patients (55.0%). Initial visual acuity was worse in patients who developed recurrence than in those who did not (P=0.024). On initial OCT images, RPE reflectivity differed significantly between patients with and without recurrence (1.75 ± 0.42 vs 1.35 ± 0.20; P=0.018), while there were no significant differences in other chorioretinal parameters, such as central retinal thickness and choroidal thickness. RPE reflectivity on OCT images may be useful for predicting the recurrence of inflammation in patients with VKH disease.

Bacillary Layer Detachment in Acute Vogt-Koyanagi-Harada Disease.

To evaluate the frequency and treatment response of eyes with bacillary layer detachment (BLD) in acute Vogt-Koyanagi-Harada (VKH) disease using spectral-domain optical coherence tomography (SD-OCT). We retrospectively reviewed the medical records of 58 eyes of acute VKH patients with at least 6 months of follow-up between January 2009 and March 2021. SD-OCT, color fundus photographs, and fluorescein angiography images were analyzed in all patients. The study included 58 eyes of 29 patients. BLD was detected in 33 of the 58 eyes (56.9%) at baseline. Mean serous retinal detachment (SRD) height was 918.50±336.64 μm in the BLD group and 215.33±167.83 μm in the group without BLD (p<0.05). A positive correlation was found between SRD height and the presence of BLD (r=0.783, p<0.05). BLD was significantly more common in patients with a baseline SRD height greater than 500 μm (p<0.05). As subfoveal central choroidal thickness (CCT) could not be measured by enhanced depth imaging-OCT at baseline due to extreme choroidal thickness in all eyes, the earliest post-treatment CCT measurements were analyzed. At the completion of pulse steroid therapy, mean CCT was 425±82.87 μm in the BLD group and 385.58±82.87 μm in the group without BLD (p=0.04). The mean time to BLD resolution was 12.88±6.5 days (range: 2-26). BLD is a common tomographic finding in eyes with acute VKH disease and can be differentiated from the associated SRD through careful SD-OCT analysis. Though it is mostly observed in patients with more serious disease, the presence of BLD has no negative effect on long-term visual function.

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.

The purpose of the study was to investigate clinical relevance of fibrin membranous structure (FMS) in the photoreceptor outer segments on spectral-domain optical coherence tomography (SD-OCT) in untreated initial-onset acute Vogt-Koyanagi-Harada (VKH) disease. Clinical charts of 39 patients (78 eyes) diagnosed with initial-onset VKH disease were retrospectively reviewed. Age, gender, period from the onset of symptoms to first visit, visual acuity (VA), intraocular pressure (IOP), anterior chamber cells, serous retinal detachment (SRD), SD-OCT findings, as well as fluorescein (FA), and indocyanine green angiography (ICGA) were collected. FMS was observed in 24 out of 39 VKH patients in either eye (61.5%). VKH patients with FMS (FMS group) were significantly younger and had the shorter period from the onset of symptoms to the first visit compared with those without FMS (non-FMS group). Mean logMAR VA and proportion of pooling of dye, mean central retinal thickness (CRT) were significantly higher in FMS group than in non-FMS group. In contrast, hyperfluorescence of the optic disc on FA was more in non-FMS group than in FMS group. Significant positive correlations between CRT and logMAR VA or IOP were only observed in the FMS group. Total amount of corticosteroids was significantly greater in FMS group than in non-FMS group. However, there were no significant differences in LogMAR VA and IOP between two groups at 6months after treatment initiation. FMS on SD-OCT is a critical feature observed in the early stage of initial-onset acute VKH disease, which is more common in younger patients and is related to the disease activity.

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

To investigate the outcomes of initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease that occurred during pregnancy. This is a retrospective case series. 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 108months (mean 35.2 ± 28.3months). 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. Primary treatment with combined systemic corticosteroids and cyclosporine in initial-onset acute uveitis associated with VKH disease was safe and effective.

Outcomes of patients with acute Vogt-Koyanagi-Harada disease treated with intravenous corticosteroid pulse followed by the slow tapering of oral corticosteroid therapy.

To elucidate the intravenous corticosteroid pulse treatment outcomes of patients with acute Vogt-Koyanagi-Harada (VKH) disease and assess the differences between patients with no inflammation worsening and those with persistent or worsening inflammation. Potential factors responsible for eyes with low visual outcomes were also investigated. We retrospectively reviewed the clinical records of patients with acute VKH disease who first visited us between 2009 and 2018 and were followed up for > 300days. Clinical characteristics, treatments, and posttreatment conditions were assessed. Patients were classified into no inflammation worsening (acute-resolved [AR]) and inflammation worsening (chronic-recurrent [CR]) groups based on conditions after 6months from disease onset. This study included 60 eyes from 30 patients (mean age: 52.7years). Patients were treated with methylprednisolone pulse followed by the slow tapering of oral prednisolone; 73% of patients developed AR and 27% CR, and the best-corrected visual acuity (BCVA) was ≥ 1.0 in 83% of eyes at 6months following the introduction of treatment. Although the total prednisolone dose was higher in patients with CR disease, no significant difference was noted in the final BCVA. Among the patients, five eyes had a final BCVA of ≤ 0.5 due to anisometropic amblyopia, diabetic maculopathy, pre-existing macular hole, epiretinal membrane, and ellipsoid zone loss. Patients with acute VKH disease treated with corticosteroid pulse appear to demonstrate good visual outcomes, including patients with CR; the majority of eyes with low visual outcomes have pre-existing conditions that explain the low vision.

Long-term changes of choroidal blood flow velocity in Vogt-Koyanagi-Harada disease.

To evaluate changes in choroidal blood flow in patients with Vogt-Koyanagi-Harada (VKH) disease after initiation of corticosteroid treatment. Fourteen patients (10 men and 4 women) with acute VKH disease followed for 2years were retrospectively reviewed; only right eyes were included in the analysis. Mean blur rate (MBR) in the macula was measured by laser speckle flowgraphy (LSFG) and central choroidal thickness (CCT) was measured by optical coherence tomography (OCT), both prior to treatment and over 2years after initiation of corticosteroid treatment. Of 14 patients included in this study, 13 received initial treatment consisting of intravenous corticosteroid pulse therapy and one patient was treated using bilateral sub-Tenon injections of triamcinolone acetonide. Mean percentage change in MBR was significantly increased after initiation of treatment compared to pretreatment values (P < 0.001). Mean CCTs were significantly decreased after initiation of treatment, compared to pretreatment thicknesses (P < 0.001). There was no significant change in either MBR change or CCT at 1month after initiation of treatment through 2years of follow-up. The mean MBR percentage change was significantly higher in eyes with sunset glow fundus (SGF) compared to eyes without SGF at 1year. With initiation of corticosteroid treatment in VKH disease patients, choroidal blood flow improved and was maintained for 2years. However, the presence of SGF should be taken into consideration when interpreting MBR results in VKH disease patients.

Elevated Serum Immunoglobulin E Levels are Associated with the Severity of Newly Diagnosed, Acute Vogt–Koyanagi–Harada Disease

ABSTRACT Objective To investigate the correlation between serum immunoglobulin E (IgE) levels and severity of Vogt–Koyanagi–Harada (VKH) disease. Methods The medical records of patients with VKH disease between 2015 and 2020 were reviewed. Serum immunoglobulins (IgA, IgE, IgG, and IgM), tumor necrosis factor α (TNFα) and C-reactive protein (CRP) were measured. Patients were divided into IgE-positive (IgE ≥ 100 IU/mL) and IgE-negative (IgE < 100 IU/mL) groups. The best-corrected visual acuity (BCVA) and macular morphologic characteristics including foveal thickness (FT), serous retinal detachment (SRD), sensory retinal thickness (SRT), central foveal thickness (CFT), cube volume (V), and cube average thickness (AT) were determined in patients in both groups. Results Of 128 patients included in the study, 35 (27.34%) patients were IgE-positive, BCVA (logMAR) was worse in the IgE-positive group. The mean CRP (P= .012) and TNFα (P≤ 0.001) levels were greater in the IgE-positive group than in the IgE-negative group. Regarding macular morphologic characteristics, FT (P= .010), SDR (P= .004), CFT (P= .008), V (P= .013), and AT (P= .006) were significantly greater in the IgE-positive group than in the IgE-negative group. Conclusions Elevated serum IgE levels were associated with more severe macular changes in patients with VKH disease. These findings suggest that IgE may be involved in the progression of VKH disease.

Pseudo-inflammatory manifestations of choroidal lymphoma resembling Vogt-Koyanagi-Harada disease: case report based on multimodal imaging

BackgroundHematologic malignancies occasionally cause serous retinal detachment (SRD); however, its pathogenesis remains unclear. Here we present the imaging characteristics of metastatic choroidal lymphoma masquerading as Vogt-Koyanagi-Harada (VKH) disease.Case presentationA 45-year-old Japanese woman was referred to our clinic because of bilateral SRD with blurred vision. Fluorescein angiography revealed multiple pinpoint leakage followed by pooling OU. Enhanced depth imaging optical coherence tomography showed marked choroidal thickening OU. Laser speckle flowgraphy detected choroidal circulation impairment OU. Although these results totally agreed with the inflammatory manifestations of acute VKH disease, indocyanine green angiography demonstrated various sizes of sharply marginated hypofluorescent lesions that seemed atypical for the finding of VKH disease, i.e., vaguely marginated hypofluorescent small dots. Cerebrospinal fluid pleocytosis was not detected. Blood tests revealed leukocytosis together with elevation of lactate dehydrogenase and soluble interleukin-2 receptor levels. Corticosteroid pulse therapy did not improve any ocular findings. Bone marrow biopsy was then performed, leading to a definite diagnosis of diffuse large B-cell lymphoma. After starting systemic chemotherapy, both SRD and choroidal thickening resolved rapidly with visual recovery. However, choroidal hypoperfusion persisted, which contrasted distinctly with the inflammatory pattern of VKH disease, i.e., the restoration of choroidal blood flow in parallel with normalization of choroidal thickness.ConclusionsOur detailed multimodal observations highlighted the differential imaging features of choroidal lymphoma despite close resemblance to VKH disease especially at the initial stage. Impaired circulation in the thickened choroid marked the pseudo-inflammatory pathogenesis of SRD due to choroidal involvement with neoplastic, but not inflammatory cells.

Textural Properties of Choriocapillaris on OCTA in Healed Inflammatory Choriocapillaropathies.

To evaluate the flow characteristics and textural properties of choriocapillaris (CC) on optical coherence tomography angiography in eyes with resolved inflammatory choriocapillaropathies and Vogt-Koyanagi-Harada (VKH) disease. A cohort of eyes with healed acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and VKH disease were included. A 3 mm × 3 mm OCT angiogram of CC was acquired and graded for flow characteristics and textural properties. This study included 16 patients. Texture was heterogeneous in all eyes in the SC and VKH groups, and in four eyes (40%) in the APMPPE group. Most of the eyes with VKH disease had severe low flow, whereas most of the SC and APMPPE eyes demonstrated mild low flow. Heal duration had a strong negative correlation with severity of CC low flow and a weak, statistically nonsignificant correlation with texture heterogeneity. Despite the resolution of active inflammation, partial CC hypoperfusion and texture disruptions persist for longer durations and may resolve in a time dependent manner. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:566-572.].

Long-term changes of retinal pigment epithelium in the eyes with Vogt-Koyanagi-Harada disease observed by adaptive optics imaging.

Purpose: To observe long-term changes of the retinal pigment epithelium (RPE) in the eyes of patients with Vogt-Koyanagi-Harada (VKH) disease using an adaptive optics (AO) fundus camera, and their correlation with the findings of spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (AF) imaging.Patients and methods: Three eyes of two patients with new-onset acute VKH disease were retrospectively studied. After the serous retinal detachment was resolved by high-dose corticosteroid treatment, the patients were examined with SD-OCT, blue-wavelength AF, near-infrared (NIR) AF, and an AO fundus camera. AO images of the macula were obtained using the rtx1TM AO fundus camera. The area around the foveal center of the hyper-reflective lesion in AO imaging was measured manually. The time at which the serous retinal detachment resolved was set as the baseline, and AO and other images were obtained every 3 to 6 months from the baseline.Results: In all three eyes, lesions with elevation or thickening of the RPE layer were observed with OCT imaging in the macula after the serous retinal detachment resolved. These lesions showed hyper-autofluorescence in NIR-AF image and hyper-reflective lesions with clear boundaries in AO image. The area of the hyper-reflective lesions of AO images in each eye showed an approximately 40% decrease at 6 months from baseline. However, the hyper-reflective lesion remained to some extent after 18 months in case 1 and 36 months in case 2.Conclusions: By using OCT, fundus autofluorescence and AO images, it was possible to observe temporal changes of RPE layer in VKH eyes noninvasively. High-resolution AO images also allow us to observe for improvements in the elevation or thickening of the RPE layer quantitatively.

Ultra-wide-field angiography findings in acute Vogt-Koyanagi-Harada disease

Background/aimsTo investigate the prevalence of abnormal central and peripheral ultra-wide-field (UWF) angiography findings, and their association with clinical features in acute Vogt-Koyanagi-Harada (VKH) disease.MethodsThis retrospective, observational study included 26 eyes...

Clinical features and visual outcomes of 111 patients with new-onset acute Vogt-Koyanagi-Harada disease treated with pulse intravenous corticosteroids

PurposeTo describe the clinical features, treatment and visual outcomes of Japanese patients with new-onset acute Vogt-Koyanagi-Harada (VKH) disease.MethodsClinical records of 111 patients who presented between 1999 and 2015 to the...

Changes in Central Macular Thickness and Retinal Nerve Fiber Layer Thickness in Eyes with Vogt-Koyanagi-Harada Disease: A 2-Year Follow-Up Study

Purpose: To investigate the central macular thickness (CMT) and retinal nerve fiber layer (RNFL) thickness in eyes with Vogt-Koyanagi-Harada (VKH) disease associated with optic disc swelling and serous retinal detachment through a 24-month follow-up period. Methods: We prospectively investigated 28 eyes of 14 treatment-naïve patients with acute VKH disease associated with optic disc swelling and serous retinal detachment and 30 eyes of 15 normal individuals to compare changes in the CMT and average RNFL thickness. Results: The CMT was significantly lower in the eyes of the VKH group at 12 and 24 months. The RNFL thickness was significantly higher in the eyes of the VKH group at the initial visit and at the 6- and 12-month follow-up visits, but no significant difference was found between the VKH group and control group at the 24-month follow-up visit. Conclusion: Significant changes in the CMT and RNFL thickness in the eyes with VKH disease were observed during the 24-month follow-up period. When diagnosing or monitoring diseases including glaucoma and neuro-ophthalmic diseases that affect the retinal thickness in patients with VKH disease, we recommend considering longitudinal changes in the retinal thickness.

Recovery of macular cone photoreceptors in Vogt-Koyanagi-Harada disease.

Our purpose was to study changes in macular cone photoreceptors in Vogt-Koyanagi-Harada (VKH) disease patients after high-dose corticosteroid treatment using an adaptive optics (AO) fundus camera. We retrospectively analyzed 16 eyes of eight patients with new-onset acute VKH disease that were studied retrospectively. After serous retinal detachment (SRD) had resolved, AO images were obtained using the rtx1™ AO fundus camera over a 12-month course. Cone counting was performed in the nasal, temporal, superior and inferior areas of the macula 0.75mm from the foveal center. A control group of 30 eyes of 30 healthy subjects was established for comparison. In the eyes with VKH disease, the mean cone densities 0.75mm from the foveal center were 11,847 at baseline (resolution of SRD), and 15,218, 16,684 and 17,686 cones/mm2, at 3, 6, and 12months later, respectively. The mean cone densities at 3, 6, and 12months were significantly increased compared to those of baseline (p=0.007, p<0.001, and p<0.001, respectively); however, they were significantly lower than that of the healthy control eyes (p<0.001). The mean cone densities at areas with a previous presence of cystoid spaces were significantly lower than those without cystoid spaces at the baseline, and at 3, 6, and 12months (p<0.001, p=0.007, p<0.001, and p=0.008, respectively). Cone densities were gradually increased after the resolution of SRD in the eyes of VKH disease patients. The presence of cystoid spaces might be a marker of severe damage to cone photoreceptors.

Early post-treatment choroidal thickness to alert sunset glow fundus in patients with Vogt-Koyanagi-Harada disease treated with systemic corticosteroids.

PurposeTo determine if early post-treatment central choroidal thickness (CCT) changes can predict sunset glow fundus (SGF) development in patients with Vogt-Koyanagi-Harada (VKH) disease treated using systemic corticosteroids.MethodsThis retrospective case series included 39 eyes of 21 treatment-naïve patients with acute VKH disease who could be followed up for more than 12 months after systemic corticosteroid therapy. The eyes were divided into two groups according to whether SGF was present or absent at 12 months (9 eyes of 5 patients versus 30 eyes of 16 patients, respectively). Using enhanced depth imaging optical coherence tomography, CCT values were measured before treatment, then at 1 week and 1 and 3 months after treatment in both groups and compared between the two groups.ResultsDevelopment of SGF was found 4–11 months after treatment. Mean post-treatment CCT decreased significantly at all examinations compared with baseline in both groups, along with resolution of serous retinal detachment. One week after treatment, mean CCT was significantly higher in eyes with SGF than in those without (P = 0.024). SGF was present at 12 months in 9 of 22 eyes with CCT values > 410 μm at 1 week after starting treatment, in contrast with none of 17 eyes with CCT ≤ 410 μm at this time (P = 0.003).ConclusionsThe current study suggested the potential validity of early post-treatment CCT as a feasible index to alert future progression to SGF in patients with VKH disease treated using systemic corticosteroids.

Changes of central macular thickness and retinal nerve fiber layer thickness in eyes with Vogt‐Koyanagi‐Harada disease: a 2‐year follow‐up study

PurposeTo prospectively investigate the change in central macular thickness (CMT) and retinal nerve fiber layer (RNFL) thickness in eyes with Vogt‐Koyanagi‐Harada (VKH) disease in through 24 months follow‐up.MethodsTwelve eyes of 6 treatment‐naïve patients with acute VKH disease associated with optic disc swelling and serous retinal detachment and 30 eyes of 15 normal individuals were enrolled to investigate the changes of CMT and average RNFL thickness. Retinal thickness were measured at first visit and months 6, 12 and 24 via spectral domain optical coherence tomography (SD‐OCT).ResultsCMT was significantly lower in the eyes of VKH group at 12 (246.58 ± 21.80 μm vs. 258.60 μm ± 15.61, p &lt; 0.05) and 24 months visit (226.67 ± 14.21 μm vs. 253.15 ± 15.84 μm, p &lt; 0.05). RNFL thickness was significantly higher in the eyes of VKH group at initial visit, 6 and 12 months follow‐up visit, but no significant difference between VKH group and control group at 24 months follow‐up visit (95.83 ± 14.85 μm vs. 97.14 ± 12.97 μm, p &gt; 0.05).ConclusionsThere were significant changes of CMT and RNFL thickness in the eyes with VKH disease during 24 months follow‐up period. Detecting or monitoring diseases including glaucoma and neuro‐ophthalmic diseases, which affect the retinal thickness in VKH patients, we recommend to consider the longitudinal change of the retinal thickness.

Mycophenolate mofetil combined with systemic corticosteroids prevents progression to chronic recurrent inflammation and development of 'sunset glow fundus' in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease.

To evaluate the effectiveness and safety of mycophenolate mofetil (MMF) as first-line therapy combined with systemic corticosteroids in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease. This prospective study included 38 patients (76 eyes). The main outcome measures were final visual acuity, corticosteroid-sparing effect, progression to chronic recurrent granulomatous uveitis and development of complications, particularly 'sunset glow fundus'. The mean follow-up period was 37.0±29.3 (range 9-120months). Visual acuity of 20/20 was achieved by 93.4% of the eyes. Corticosteroid-sparing effect was achieved in all patients. The mean interval between starting treatment and tapering to 10mg or less daily was 3.8±1.3months (range 3-7months). Twenty-two patients (57.9%) discontinued treatment without relapse of inflammation. The mean time observed off of treatment was 28.1±19.6months (range 1-60months). None of the eyes progressed to chronic recurrent granulomatous uveitis. The ocular complications encountered were glaucoma in two eyes (2.6%) and cataract in five eyes (6.6%). None of the eyes developed 'sunset glow fundus', and none of the patients developed any systemic adverse events associated with the treatment. Use of MMF as first-line therapy combined with systemic corticosteroids in patients with initial-onset acute VKH disease prevents progression to chronic recurrent granulomatous inflammation and development of 'sunset glow fundus'.

The Role of Optical Coherence Tomography Angiography in the Diagnosis and Management of Acute Vogt–Koyanagi–Harada Disease

ABSTRACTPurpose: To report the imaging characteristics of acute Vogt–Koyanagi–Harada (VKH) disease using optical coherence tomography angiography (OCTA).Methods: In this prospective study, patients with acute VKH (n = 10; mean age: 30.5 ± 13.43 years) underwent multimodal imaging (baseline and follow-up) using fundus photography, fluorescein angiography (FA), indocyanine green angiography (ICGA), OCT, and OCTA. The OCTA images were analyzed to assess the retinochoroidal vasculature and compared with other imaging techniques.Results: During the active stage, all eyes showed multiple foci of choriocapillaris flow void that correlated with ICGA. These foci decreased in number and size after initiation of therapy. In one patient, flow void areas reappeared after cessation of therapy without any detectable change on ICGA. This patient soon developed clinical recurrence requiring re-initiation of immunosuppression.Conclusions: OCTA allows high-resolution imaging of inflammatory foci suggestive of choriocapillaris hypoperfusion in acute VKH disease non-invasively. OCTA may be very helpful in the follow-up of such patients.