Vogt-Koyanagi-Harada Research Articles

Vogt-Koyanagi-Harada Disease Presented with Bilateral Optic Disc Edema

Vogt-Koyanagi-Harada (VKH) disease is a multisystem inflammatory disease that occurs when body tissues including melanocytes are damaged by autoimmunity. The typical symptoms of VKH are bilateral multifocal uveitis and serous retinal detachment. Our patient was misdiagnosed as idiopathic intracranial hypertension because there were no other characteristic neurological symptoms other than bilateral optic disc edema. However, intracranial pressure was normal and pleocytosis was found, so VKH disease could be diagnosed. Bilateral optic disc edema may be a initial manifestation of VKH disease.

Posterior segment optical coherence tomography: A diagnostic aid in posterior uveitis

Background: Patients with posterior uveitis can develop vision-threatening complications. Optical coherence tomography (OCT) plays an important role in the diagnosis and management of these complications. Aims: The objectives of this study were to describe different retinal morphological characteristics presenting on OCT in patients with posterior uveitis, with an aim to facilitate early diagnosis to initiate specific treatment and also to observe the response to treatment. Materials and Methods: A cross-sectional prospective non-randomized study was undertaken at a tertiary eye care hospital. The study included 30 eyes of 30 patients with posterior uveitis between the period of May 2016 and May 2018. All subjects underwent a set of systemic investigations for etiological diagnosis and complete ophthalmic examination, including OCT (Zeiss Cirrus machine (model number 5000)] imaging). Data were collected and analyzed. A P value of <0.05 was considered statistically significant. Results: The mean patient age was 33.1 ± 4.2 years. Infectious etiology was diagnosed in 13 patients (43.33%) of which toxoplasma chorioretinitis (36.66%) was the most common infection. Among 17 patients with noninfectious uveitis (56.66%), Vogt-Koyanagi-Harada (VKH) disease and multifocal choroiditis (16.67% each) were the most common causes. RPE-Bruch’s membrane abnormalities were seen in 96.67% of patients. Subretinal detachment (26.67%) was the most common type of macular edema on OCT. Significant differences in central foveal thickness posttreatment were noted in cases with VKH syndrome and toxoplasma retinochoroiditis (P < 0.05). Conclusion: OCT is a useful tool complementary to conventional fundus photography and fluorescein angiography in patients with posterior uveitis.

CHANGES IN SCLERAL THICKNESS IN THE ACUTE PHASE OF VOGT-KOYANAGI-HARADA DISEASE.

To evaluate changes in scleral thickness in Vogt-Koyanagi-Harada (VKH) disease. This study included 34 eyes of 17 treatment-naïve patients with acute-phase VKH disease. Scleral thickness and the presence of ciliochoroidal effusion were examined using anterior segment optical coherence tomography at baseline and 1 week, 2 weeks, and 12 weeks after the start of corticosteroid treatment. Scleral thickness was measured 6 mm posterior to the scleral spur in four directions. Twenty-eight eyes (82.4%) initially had ciliochoroidal effusion, but this rapidly decreased to nine eyes (26.5%) after 1 week. The sclera with ciliochoroidal effusion became thinner from baseline to 1 week at the superior (400.2 ± 46.9-353.5 ± 47.9 µm), temporal (428.4 ± 53.6-387.8 ± 56.1 µm), inferior (451.5 ± 71.0-400.5 ± 50.5 µm), and nasal (452.4 ± 78.0-407.6 ± 62.9 µm) points (P < 0.01 for all), and no further changes were observed. The sclera without ciliochoroidal effusion remained unchanged. In VKH disease, eyes with ciliochoroidal effusion exhibited the maximum scleral thickness during the acute phase. This thickening responded rapidly to treatment and became thinner within 1 week. Inflammation in VKH disease may affect not only the choroid but also the sclera.

Enhanced depth imaging optical coherence tomography features of two types of Vogt-Koyanagi-Harada disease: fuzzy or lost pattern of the choroidal vasculature is of diagnostic value.

This study aimed to compare enhanced depth imaging optical coherence tomography (EDI-OCT) features of exudative retinal detachment (ERD) type and optic disc (OD) swelling type Vogt-Koyanagi-Harada (VKH) disease. Hospitalized VKH patients were retrospectively reviewed and classified into the ERD type and the OD swelling type. The EDI-OCT features were then analyzed. The study included 32 ERD type and 15 OD swelling type VKH patients at the acute uveitis stage. The interval between the onset of ocular symptoms and the start of treatment in OD swelling type VKH disease was significantly longer compared to the ERD type (p < 0.001). A fuzzy or lost pattern of the choroidal vasculature was observed in 100% of VKH patients of both types. Moreover, high frequencies (greater than or equal to 50%) of fluctuations in the internal limiting membrane, interdigitation zone disruption, ERD, retinal pigment epithelium (RPE) folds, and ellipsoid zone disruption were observed in both types. Patients with OD swelling type VKH disease exhibited higher frequencies of OD swelling and hyperreflective substances above the RPE (p < 0.001 and p = 0.003, respectively), with lower frequencies of ERD and bacillary layer detachment (p = 0.012 and p < 0.001, respectively). At the convalescence stage, changes in the EDI-OCT images of 10 ERD type and 5 OD swelling type VKH patients were analyzed. The frequencies of the OCT features decreased with similar trends in both types of VKH disease. Although ERD type and OD swelling type VKH disease have their own unique characteristics, they share common EDI-OCT features. The Fuzzy or lost pattern of the choroidal vasculature that indicates choroidal inflammation may serve as a diagnostic aid for VKH disease, especially for the OD swelling type and the early-stage ERD type.

Vogt-Koyanagi-Harada Disease: A Narrative Review.

Vogt-Koyanagi-Harada (VKH) disease is an idiopathic immune-related sickness that affects multiple systems and melanocytes in organs such as the uvea, ear, and meninges. The primary cause of activity is cellular immunological responses. Vogt-Koyanagi disease is identified primarily by skin abnormalities and anterior uveitis. Harada's illness is distinguished by neurological symptoms and exudative retinal detachments, which are associated with the HLA-DR4 and HLA-DRw53 genes.Pigmented races, such as Hispanics and Native Americans, are more likely to have VKH disease. Clinical features are blurred vision, floaters, alopecia, vitiligo, diffuse choroidal inflammation with disc edema, and exudative retinal detachment. Differential diagnoses include posterior scleritis, uveal effusion syndrome, central serous chorioretinopathy, and sympathetic ophthalmitis. The investigations used are optical coherence tomography (OCT), fundus fluorescein angiography (FA), and B-scan ultrasonography (USG). Treatment is done by using systemic steroids, cycloplegics, and immunosuppressants.

A metagenome-wide association study of gut microbiome in patients with AMD, ASD, RA, T2D & VKH diseases

Clinical studies have already illustrated the associations between gut microbes and diseases, yet fundamental questions remain unclear that how we can universalize this knowledge. Considering the important role of human gut microbial composition in maintaining overall health, it is important to understand the microbial diversity and altered disease conditions of the human gut. Metagenomics provides a way to analyze and understand the microbes and their role in a community manner. It provides qualitative as well as quantitative measurements, in terms of relative abundance. Various studies are already going on to find out the association between microbes and diseases; still, the mined knowledge is limited. Considering the current scenario, using the targeted metagenomics approach, we analyzed the gut microbiome of 99 samples from healthy and diseased individuals. Our metagenomic analysis mainly targeted five diseased microbiomes (i.e., Age-related macular degeneration, Autism spectrum disorder, Rheumatoid arthritis, Type 2 diabetes and Vogt-Koyanagi harada), with compare to healthy microbiome, and reported disease-associated microbiome shift in different conditions.

Comprehensive Proteomic Profiling of Aqueous Humor in Idiopathic Uveitis and Vogt-Koyanagi-Harada Syndrome.

Idiopathic uveitis (IU) and Vogt-Koyanagi-Harada (VKH) syndrome are common types of uveitis. However, the exact pathological mechanisms of IU and VKH remain unclear. Proteomic analysis of aqueous humor (AH), the most easily accessible intraocular fluid and a key site of uveitis development, may reveal potential biomarkers and elucidate uveitis pathogenesis. In this study, 44 AH samples, including 12 IU cases, 16 VKH cases, and 16 controls, were subjected to label-free quantitative proteomic analysis. We identified 557 proteins from a comprehensive spectral library of 634 proteins across all samples. The AH proteomic profiles of the IU and VKH groups were different from those of the control group. Differential analysis revealed a shared pattern of extracellular matrix disruption and downregulation of retinal cellular proteins in the IU and VKH groups. Enrichment analysis revealed a protein composition indicative of inflammation in the AH of the IU and VKH groups but not in that of the control group. In the IU and VKH groups, innate immunity played an important role, as indicated by complement cascade activation and overexpression of innate immune cell markers. Extreme gradient boosting (XGBoost), an efficient and robust machine learning algorithm, was subsequently used to screen potential biomarkers for classifying the IU, VKH, and control groups. Transferrin and complement factor B were deemed the most important and represent a promising biomarker panel. These proteins were validated by high-resolution multiple reaction monitoring (HR-MRM) in an independent validation cohort. A classification decision tree was subsequently built for the diagnosis. Our findings further the understanding of the underlying molecular mechanisms in IU and VKH and facilitate the development of potential therapeutic and diagnostic strategies.

Literature data mining on the current research status of uveitis in China

Objective: To analyze the current research status of uveitis in China. Methods: It was a bibliometric analysis study. Using search formulas covering uveitis and its multiple subtypes, uveitis-related literature in English with publication dates from 2013 to 2022 was retrieved in Web of Science core databases through certain search strategies. This study used the latent Dirichlet allocation (LDA) algorithm to build topic models and analyzed the trends of research topics in recent years. Bibliometric analysis was used to analyze and visualize the bibliometric indicators (e.g., number of publications, citations, and H-index) of the included literature using tools such as VOSviewer software. Results: Over the past decade, China has published 1 657 papers on uveitis, ranking second globally. However, there is still room for improvement in terms of the H-index (58) and citation (12.28 per publication). Countries such as the USA (43.04%) and the United Kingdom (62.54%) were engaged in more international collaboration. We identified ten optimal LDA topics for uveitis literature in China such as immunotherapy, Behçet's disease, and Vogt-Koyanagi-Harada syndrome. Research on uveitis in China was mostly published in Ocular Immunology and Inflammation (92). Conclusions: China has made remarkable progress in uveitis research. Nonetheless, there is still untapped potential to enhance our global academic influence. It is encouraged to promote international collaborations, harness our expertise in areas like Behçet's disease and VKH syndrome, and publish our scientific achievements in high-impact journals.

Blood Whispers: Exploring Hematologic Indicators for Diagnosing and Predicting Severity of Vogt-Koyanagi-Harada Syndrome

ABSTRACT Purpose To investigate the association of clinical findings and indocyanine green angiography (ICGA) score with inflammatory markers derived from complete blood count (CBC) parameters in patients with Vogt-Koyanagi-Harada (VKH) to determine the diagnostic and predictive role. Methods Demographic characteristics, presenting complaints, ocular findings, optical coherence tomography findings, ICGA scores and best corrected visual acuity were recorded in treatment-naive VKH patients at presentation. Patients were divided into two groups as acute stage and chronic recurrent stage. CBC parameters were noted in patients at presentation and healthy controls (HC, n = 25). Neutrophil-lymphocyte-platelet-monocyte counts, neutrophil/lymphocyte (NLR), platelet/lymphocyte (PLR), monocyte/lymphocyte and systemic immune-inflammation index (SII) were recorded. The association between these markers and clinical severity were evaluated. Results Thirty-two patients with VKH (23 females/9 males) with a mean age of 34.1 ± 14.6 years were included in the study. There was an increase in neutrophil count, NLR and SII in patients with VKH compared to HC (p < 0.001). The cut-off values for these three parameters were 4.37, 2.24 and 562.35, respectively. Twenty-six patients presented in the acute stage and six patients presented in the chronic recurrent stage. Choroidal thickness, early stromal hyperfluorescence and total ICGA scores were higher in patients presenting in the acute stage (p < 0.001, 0.001 and 0.025, respectively). Patients with higher disease severity at presentation were treated earlier. Early stromal vessel hyperfluorescence and choroidal vasculitis scores were correlated with decreased lymphocyte count, increased NLR, PLR and SII (p < 0.05). Conclusion CBC-derived inflammatory parameters indicate that VKH is a systemic inflammation. These parameters can be used in the diagnosis and determination of disease severity of VKH.

A unique case of VKH in HIV patient

A 49-year-female with known human immunodeficiency virus on highly active antiretroviral therapy presented with diminution of vision and mild pain in both eyes of 2 weeks duration, preceded by alopecia, headache, and tinnitus. Her best corrected visual acuity was 6/60 in both eyes, with anterior segment inflammation and multiple pockets of neurosensory detachments in the fundus bilaterally. Systemic workup done to rule out other causes of uveitis was negative. A diagnosis of complete Vogt–Koyanagi–Harada syndrome was made and treated with systemic corticosteroids, which resolved upon treatment, with improvement in visual acuity to 6/9P.

Cataract Surgery in Vogt — Koyanagi — Harada Syndrome (Clinical Case)

The article describes a clinical case of the Vogt — Koyanagi — Harada syndrome with an ophthalmic manifestations: bilateral granulomatous panuveitis with funnel-shaped serous retinal detachment complicated by cataracts, clinical manifestations in the form of alopecia and poliosis, as well as the results of phacoemulsification with IOL implantation.

Treatment of uveitis and scleritis patients in Malaysia.

To determine the common causes and visual outcome after treatment among uveitis and scleritis patients. This is a retrospective cohort observational study. All consecutive clinical records of patients with newly diagnosed uveitis and scleritis over a 4-year period, from Jan. 1, 2017 to Dec. 31, 2020, were analysed. Data was collected at the presentation and included a follow-up period of one year. A total of 288 patients were recruited during the study period. Anterior uveitis was the most common anatomical diagnosis (50.0%) followed by panuveitis (25.0%), scleritis (13.5%), posterior uveitis (6.9%), and intermediate uveitis (4.5%). Viral Herpes was the most common cause of infectious cases, while Vogt-Koyanagi-Harada (VKH) disease and human leucocyte antigen (HLA) B27 spondyloarthropathy were the leading causes of identifiable non-infectious cases. Majority of patients presented with unilateral, non-granulomatous uveitis with an absence of hypopyon. Anatomical locations like posterior uveitis and panuveitis, and visual acuity worse than 3/60 at presentation were the factors associated with poor visual outcomes (P<0.05). About 60% of patients had an identifiable cause for the uveitis and scleritis, with nearly equal distribution of infectious (n=85, 29.5%) and non-infectious causes (n=84, 29.2%). About 14.5% of patients were clinically blind at 1y of follow-up. The most common complication in our uveitis patients was glaucoma (47.5%), followed by cystoid macula oedema (18.9%) and cataract (13.9%). Uveitis and scleritis are important causes of ocular morbidity. They are potentially blinding diseases which can have a good outcome if diagnosed and treated early.

A Case of Asymmetrical Vogt-Koyanagi-Harada Disease in a Patient with Ota Nevus Following Laser Treatment

Purpose: To report a case of Vogt-Koyanagi-Harada (VKH) disease exhibiting asymmetric symptoms, predominantly in the affected eye, aside from the bilateral findings noted in indocyanine green angiography (ICG) in a patient who had undergone treatment for nevus of Ota.Case summary: A 33-year-old male presented with decreased visual acuity, ocular pain in the right eye, and a 3-week history of headache. The patient had undergone two laser treatments at 1-month intervals to address a nevus of Ota on the upper and lower eyelids and the maxillary area of the right side. He had no notable medical history. Fundus examination and optical coherence tomography indicated multiple serous retinal detachments. ICG displayed multiple hyperfluorescent spots in both eyes. Given these findings, the patient was diagnosed with VKH disease, predominantly affecting the right eye. Human leukocyte antigen (HLA) testing revealed the presence of the HLA-DRB1*09 and HLA-DQB1*03 (DQ8) alleles. The patient initially received high-dose intravenous steroid therapy, later transitioning to oral steroids. After a month of treatment, the serous retinal detachments had resolved and his vision improved. No signs of recurrence were evident during a 6-month follow-up.Conclusions: VKH disease can manifest asymmetrically, especially in the eye affected after laser treatment for nevus of Ota. Despite potential monocular presentation, it is crucial to assess for binocular involvement through comprehensive examinations.

Vogt-Koyanagi-Harada disease developed during chemotherapy for Hodgkin lymphoma: a case report

BackgroundOcular manifestations are known for non-Hodgkin lymphoma, but are rare for Hodgkin lymphoma. We report a case of Vogt-Koyanagi-Harada (VKH) disease presenting as serous retinal detachment and uveitis in both eyes in a child undergoing chemotherapy for Hodgkin lymphoma.Case presentationThe patient was a 7-year-old boy with stage IIB Hodgkin lymphoma (nodular lymphocyte predominant type) who was undergoing chemotherapy, including 2 cycles of the OEPA regimen and 1 cycle of the COPDAC regimen. Two days after the end of the COPDAC regimen, the patient complained of headache and of blurred and decreased vision in both eyes. On the basis of optic symptoms, such as uveitis and serous retinal detachment in both eyes, increased cell counts in cerebrospinal fluid, and positivity for human leukocyte antigen (HLA)-DR4 in peripheral blood cells, incomplete VKH disease was diagnosed. Intravenous treatment with high-dose prednisolone (60mg/m2/day) for 7 days improved both visual acuity and serous retinal detachment and enabled the remains of the COPDAC chemotherapy cycle to be administered. With prednisolone treatment, visual acuity improved from 20/500 to 20/20 in the right eye and from 20/63 to 20/25 in the left eye. Because multiple vitiligo lesions later appeared in the abdomen, complete VKH disease was finally diagnosed.ConclusionThe onset of VKH disease occurred during chemotherapy for Hodgkin lymphoma. The patient was HLA-DR4-positive and might have had a predisposition to develop autoimmune diseases, including VKH disease. However, the anticancer drugs administered to this patient have not been reported to cause uveitis. Whether Hodgkin lymphoma triggered the development of VKH remains unclear. Early diagnosis of VKH disease and prompt treatment with high-dose prednisone enabled the patient to maintain good visual function despite chemotherapy for Hodgkin lymphoma.

Pretreatment ocular blood flow and retinal oxygen metabolism inthe acute uveitic phase is associated with final outcome in Vogt-Koyanagi-Harada disease.

To investigate the association between pretreatment blood flow velocity in the choroid and optic nerve head (ONH) and retinal oxygen metabolism in the acute uveitic phase and the development of 'sunset glow fundus' in Vogt-Koyanagi-Harada (VKH) disease. Retrospective analysis of 41 patients (82 eyes). Laser speckle flowgraphy and retinal oximetry measurements were performed at the presentation. The main outcome measure was the development of 'sunset glow fundus'. Twenty patients (40 eyes) presented in the phase preceding anterior segment inflammation (early presentation), and 21 patients (42 eyes) presented with anterior segment inflammation (late presentation). In ONH, mean blur rate (MBR)-vessel, representing blood flow velocity in retinal vessels, was significantly lower in the late presentation group, while choroidal MBR was not significantly different. The late presentation group had significantly lower oxygen saturation in retinal venules, a higher arteriovenous oxygen saturation difference and a smaller calibre of retinal arterioles compared with the early presentation group. Eyes that subsequently developed 'sunset glow fundus' had significantly lower ONH MBR-vessels, lower oxygen saturation in retinal venules, a higher arteriovenous oxygen saturation difference and a smaller calibre of retinal arterioles compared with eyes without 'sunset glow fundus'. ONH MBR-vessel had a significant negative correlation with arteriovenous oxygen saturation difference and a significant positive correlation with calibre of retinal arterioles. In the acute uveitic phase of VKH disease, the development of 'sunset glow fundus' is associated with pretreatment reduced retinal blood flow velocity, calibre of retinal arterioles and oxygen saturation in retinal venules, as well as an increased arteriovenous oxygen saturation difference.

Sympathetic ophthalmia or Vogt–Koyanagi–Harada disease: Don’t judge a book by its cover

Abstract: A 17-year-old female patient of Asian origin presented to the Ocular Immunology Unit of Reggio Emilia Hospital in July 2017, complaining of nausea, vomiting, low-grade fever, tinnitus, and headache going on for 3 days, followed by the appearance of blurred vision in the left eye. Three months before (April 2017) she had a history of penetrating keratoplasty in the right eye for a diagnosis of Acanthamoeba keratitis unresponsive to antiamoebic therapy. The clinical examination exhibited a picture of bilateral panuveitis with papillitis and exudative detachment of the retinal neuroepithelium. The diagnostic workup excluded a possible infectious etiology and showed the positivity of the human leukocyte antigen-DR4. Magnetic resonance imaging showed leptomeningeal inflammatory involvement and lumbar puncture revealed lymphocytic pleocytosis. Considering the history of trauma, Vogt–Koyanagi–Harada disease was ruled out and the diagnosis of sympathetic ophthalmia was made. The patient was treated with topical and oral steroids combined with mycophenolate mofetil for long-term control of the disease. The subsequent 18-month follow-up showed an excellent clinical response with a marked improvement in the ocular findings.

A Novel Risk Stratification-Based Immunomodulatory Treatment Strategy for Vogt-Koyanagi-Harada Disease

PurposeTo develop a more tailored immunomodulatory treatment (IMT) strategy based on a novel two-arm risk stratification system in Vogt-Koyanagi-Harada (VKH) patients. DesignA retrospective clinical cohort study. Methods79 VKH patients in the acute stage were stratified into low (n = 58) and high-risk (n = 21) groups based on their exposure to risk factors. They were treated with oral glucocorticoids (GCs) plus pro re nata (PRN) or first-line IMT. Best corrected visual acuity (BCVA), sunset glow fundus (SGF) occurrence, relapse rate, and systemic adverse events were evaluated during follow-up. ResultsCompared with the low-risk group, the high-risk showed poorer BCVA at baseline (estimated difference, 0.51; 95% CI, 0.30-0.78; p < 0.001) and 6-month follow-up (estimated difference, 0.08; 95% CI, 0.00-0.08; p = 0.006), higher incidence of SGF at 12 months (52% vs 28%; RR, 1.9; 95% CI, 1.1-3.4; p = 0.040), and higher relapse rate at 6 months (24% vs 5%; RR, 4.6; 95% CI, 1.2-17.5; p = 0.028) and 12 months (52% vs 12%; RR, 4.4; 95% CI, 1.9-9.7; p < 0.001). In the low-risk cohort, no significant difference between the two IMT strategies was observed in primary outcomes. In the high-risk cohort, patients with the immediate IMT showed better BCVA (estimated difference, -0.20; 95% CI, -0.3 to -0.08; p = 0.007), lower incidence of SGF (27% vs 80%; RR, 0.3; 95% CI, 0.1-0.9; p = 0.030), and lower relapse rate (27% vs 80%; RR, 0.3; 95% CI, 0.1-0.9; p = 0.030) compared with the PRN regimen. Moreover, the immediate IMT regimen had a higher frequency of systemic adverse events than the PRN regimen (47% vs 7%; RR, 7.1; 95% CI, 2.5-20.4; p < 0.001). ConclusionsHigh-risk stratification at baseline was associated with poor prognosis. The immediate IMT regimen was only beneficial for high-risk VKH patients regarding visual outcome, SGF, and relapse rate. This study suggests a potential need for a customized IMT strategy for VKH patients.

EARLY DIAGNOSIS AND TREATMENT OF VOGT-KOYANAGI-HARADA SYNDROME WITH OPTIC DISC SWELLING : A CASE REPORT

Introduction : Vogt-Koyanagi-Harada (VKH) syndrome is characterized as a multisystem autoimmune disease that affects the pigmented tissues of the eye, auditory system, skin, and central nervous system. Optic disc swelling is a common finding during the prodromal and acute uveitis stage and must be considered seriously.&#x0D; Case Illustration : A 41-year-old woman presented with blurred vision in both eyes in the last month, accompanied by headache, nausea, and vomiting. There is no history of penetrating ocular trauma or surgery. Examination findings for visual acuity were light perception, bilateral posterior synechia, optic disc swelling, and serous retinal detachment. A steroid injection was given due to swelling of the optic disc, and four months later, the visual acuity improved to 20/120 and 20/60, respectively, and optic disc swelling and serous retinal detachment decreased.&#x0D; Discussion : In the acute uveitic stage, VKH can be presented with bilateral optic disc edema and various degrees of serous retinal detachment. The key to the management of VKH is early diagnosis and systemic corticosteroid in the acute uveitis stage, otherwise, improper and inadequate treatment can lead to chronic recurrent VKH disease. Our patient presented with acute uveitis and was treated promptly and adequately.&#x0D; Conclusion : Optic disc swelling may occur in VKH and worsen the visual prognosis, early diagnosis and treatment of VKH syndrome focused on optic disc swelling can improve the visual prognosis.

METHOTREXATE FOR REBOUND PHENOMENON OF VOGT-KOYANAGI-HARADA IN JUVENILE: A RARE CASE REPORT

Introduction: Vogt-Koyanagi-Harada (VKH), an auto-immune disorder driven by melanocyte antigen, is rare in children. The mainstay treatments are corticosteroid and immunomodulators. Methotrexate is one of immunomodulator that widely used due to its effectiveness with minimal side effects. Case Report: A 9-years-old boy get diagnosed Vogt-Koyanagi-Harada. His best corrected visual acuities (BCVA) were 20/400 in both eyes. Anterior segment examination showed granulomatous uveitis. Posterior segment examination revealed optic disk swelling, Dallen Fuch’s nodule and exudative retinal detachment. We also found poliosis and vitiligo in his lips. He had been previously treated with topical corticosteroids, oral corticosteroids and topical cycloplegics. The dose was tapered every month. After taking medication for 5 months with the last dose oral corticosteroid 16 mg/day and topical corticosteroid every 8 hours, his BCVA recovered to 20/25 RE and 20/40 LE. During the treatment time, the patient discontinued the medication due to parental disobedience and then came with decrease of vision. Based on the consideration of rebound phenomenon and the use of maximum dose of corticosteroids, we consulted the patient to the pediatrician, and decided to give methotrexate as an immunosuppressant. After receiving 15 mg/week of methotrexate for 3 months, inflammation calmed down but unfortunately the vision did not improve. Discussion: In children, the main treatment for VKH disease is high-dose corticosteroids. Considering the side effects of prolonged systemic corticosteroid therapy, methotrexate become the first line of corticosteroid sparing agent, especially in chronic stage. Conclusion: Methotrexate can be an effective treatment option for rebound VKH. Keywords: Juvenile Vogt-Koyanagi-Harada, Steroid Sparing Agents, Rebound Phenomenon, Methotrexate.

Vogt-Koyanagi-Harada Disease: The Clinical Spectrum and Management of Case Series in a Tertiary Eye Centre in Northern Part Of Malaysia.

We present the clinical spectrum, the initial clinical presentation with management trends in treating 14 Vogt-Koyanagi-Harada (VKH) disease cases in a tertiary center in the Northern part of Malaysia. There were 14 cases of Vogt-Koyanagi-Harada (VKH) disease retrospectively reviewed over five years (from 2015 to 2020). The mean age at presentation was 37.7 years (range 21-64 years), with female predominance (85.7%). All cases presented with acute uveitic stage and bilateral eye involvement. Of them, 11 (78.6%) were probable VKH, and three (21.4%) were incomplete VKH. All patients attended with acute panuveitis at first presentation. The main posterior segment involvement was disc edema in 57.1% (16 out of 28 eyes) and exudative retinal detachment (ERD) in 35.7% (10 out of 28 eyes). Most of them presented with blindness (3/60 and worse) and moderate visual impair- ment (6/18-6/60); 35.71% each, followed by mild visual impairment (6/12-6/18), and severe visual impairment (6/60-3/60); 7.1% each. Ten patients (71.4%) required combination second-line immunomodulatory treatment during subsequent visits, and only four patients (28.6%) responded well to corticosteroid therapy. Most of the cases achieved no visual impairment (64.3%), followed by mild visual impairment (21.4%) and moderate visual impairment (14.3%), and none were severe or blind at the end of follow-up. VKH is a potentially blinding illness if there is inadequate control of the disease in the acute stage. Most of our patients achievedgood visual outcomes with early immunomodulatory treatment and systemic corticosteroids.