Purpose: To report a case of acute Vogt-Koyanagi-Harada (VKH) disease with unilateral clinical manifestations followed by late fellow eye involvement.
Case Report: This case report reviews the 12-month follow-up observation of a 44-year old woman who presented to the emergency department with unilateral progressive and painless visual blurring. Ophthalmoscopic findings, best-corrected visual acuity (BCVA), fluorescein angiography (FAG), enhanced-depth optical coherence tomography (EDIOCT), indocyanine green angiography, and response to treatment were evaluated. Her BCVA was 20/50 (logMAR: 0.4) in the right eye and 20/20 (logMAR: 0) in the left eye. Eye examination revealed optic disc swelling and multiple serous retinal detachments in the right eye and a normal left eye. She had headache, dysacusia, and mild hearing problems. Her past ocular and drug histories were unremarkable. Retinal imaging revealed characteristic features of VKH in the right eye. All laboratory testing results were inconclusive. VA and OCT findings significantly improved following the treatment with methylprednisolone 1 g/day continued by tapering dose of oral prednisolone. Two months after the presentation and during prednisolone tapering, VA of the left eye decreased and fundus examination revealed multiple serous retinal detachments in this eye.
Conclusion: Ophthalmologists should recognize unilateral and asymmetrical VKH disease with subtle systemic involvement

Dourandish, Maryam

Maryam Hosseini, Seyedeh

Mazouchi, Marjan

English

KnE Publishing Platform

Case ReportUnilateral Presentation of Vogt-Koyanagi-HaradaSyndromeSeyedeh Maryam Hosseini1, MD; Maryam Dourandish1, MD; Marjan Mazouchi2, MD1Eye Research Center, Mashhad University of Medical Sciences, Mashhad, Iran2Eye Research Center, Matini Eye Hospital, Kashan University of Medical Sciences, Kashan, IranORCID:Seyedeh Maryam Hosseini: https://orcid.org/0000-0001-9804-0390Maryam Dourandish: https://orcid.org/0000-0001-9804-0390AbstractPurpose: To report a case of acute Vogt-Koyanagi-Harada (VKH) disease with unilateralclinical manifestations followed by late fellow eye involvement.Case Report: This case report reviews the 12-month follow-up observation of a 44-yearold womanwho presented to the emergency department with unilateral progressive andpainless visual blurring. Ophthalmoscopic findings, best-corrected visual acuity (BCVA),fluorescein angiography (FAG), enhanced-depth optical coherence tomography (EDI-OCT), indocyanine green angiography, and response to treatment were evaluated. HerBCVA was 20/50 (logMAR: 0.4) in the right eye and 20/20 (logMAR: 0) in the left eye.Eye examination revealed optic disc swelling and multiple serous retinal detachmentsin the right eye and a normal left eye. She had headache, dysacusia, and mildhearing problem. Her past ocular and drug histories were unremarkable. Retinal imagingrevealed characteristic features of VKH in the right eye. All laboratory testing resultswere inconclusive. VA and OCT findings significantly improved following the treatmentwith methylprednisolone 1 g/day continued by tapering dose of oral prednisolone. Twomonths after the presentation and during prednisolone tapering, VA of the left eyedecreased and fundus examination revealed multiple serous retinal detachments in thiseye.Conclusion: Ophthalmologists should recognize unilateral and asymmetrical VKHdisease with subtle systemic involvement.Keywords: Asymmetric VKH; Enhanced-Depth Optical Coherence Tomography; IndocyanineGreen Angiography; Vogt-Koyanagi-Harada DiseaseJ Ophthalmic Vis Res 2020; 15 (1): 99–103Correspondence to:Maryam Dourandish, MD. Eye Research Center, Khatam-al-Anbia Eye Hospital, Abutaleb Junction, Gharani Blvd.,Mashhad University of Medical Sciences, Mashhad91959, Iran.E-mail: Maryam.dourandeesh.dl@gmail.comReceived: 05-02-2018 Accepted: 20-08-2018Access this article onlineWebsite: https://knepublishing.com/index.php/JOVRDOI: 10.18502/jovr.v15i1.5954INTRODUCTIONVogt-Koyanagi-Harada disease (VKH) is a rare,chronic, inflammatory granulomatous multisystemThis is an open access journal, and articles are distributed under the terms ofthe Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, whichallows others to remix, tweak, and build upon the work non-commercially, aslong as appropriate credit is given and the new creations are licensed under theidentical terms.How to cite this article: Hosseini SM, Dourandish M, Mazouchi M. Vogt-Koyanagi-Harada with Unilateral Clinical Presentation: A Case Report. JOphthalmic Vis Res 2020;15:99–103.© 2020 JOURNAL OF OPHTHALMIC AND VISION RESEARCH | PUBLISHED BY KNOWLEDGE E 99A Case of Unilateral VKH Syndrome; Hosseini et aldisorder of unknown cause that is characterized bybilateral panuveitis, iridocyclitis, exudative retinaldetachment, and optic disc swelling. The disorderaffects melanocyte-rich tissues, including the eyes,auditory system, meninges, skin, and hair.[1–5]The diagnosis of VKH is based on the RevisedDiagnostic Criteria (RDC) of 2001.[6, 7] The diseaseis classified into three categories: complete, incom-plete, and probable VKH.[6]The disease is divided into four consecutivestages, namely prodromic, acute uveitic, convales-cent, and chronic.[7] Approximately 17–73%of casesprogress to chronic stage.[8] It usually affects botheyes simultaneously.[1, 6] However, unilateral ocularinvolvement at onset, followed by later involvementof the second eye in 2–3 weeks is reported in 30%of the cases.[7, 9] Reports of long-term follow-up inpatients with unilateral ocular disease are few.[8–11]Ocular imaging modalities, including fluores-cein (FA) and indocyanine angiography (ICGA);optical coherence tomography (OCT), especiallyenhanced-depth imaging OCT (EDI-OCT); andultrasonographic findings are useful in the diagno-sis and follow-up of patients.[6]Many reports have indicated that patients maynot manifest all diagnostic criteria but seem to havethe disease.[1] Herein, we report an interesting caseof clinically unilateral probable VKHwith subclinicalfellow eye involvement on ICGA, which progressedto clinical involvement later.CASE PRESENTATIONA 44-year-old woman with progressive and pain-less visual blurring of the right eye for twodays presented to the emergency departmentof the Khatam-al-Anbia Eye Hospital, affiliatedto the Mashhad University of Medical Sciences,Mashhad, Iran. She had no history of ocularsurgery or trauma. Her medical, ocular, and drughistories were unremarkable. The best-correctedvisual acuities (BCVAs) of the right and left eyeswere 20/50 (logMAR: 0.4) and 20/20 (logMAR: 0),respectively. Her slit-lamp examination result wasnormal. Intraocular pressure was 14 mm Hg inboth eyes. Fundus examination revealed optic discswelling and multiple serous retinal detachmentsin the right eye and normal left retina. Retinalimaging revealed characteristic features of VKH inthe right eye. FA showed focal areas of delayedchoroidal perfusion, multifocal areas of pinpointleakage, pooling, and optic nerve staining (Figures1 and 2). ICGA disclosed early hyper-cyanescenceof the stromal choroidal vessels, which led tolate hyper-cyanescence in both eyes and multiplehypofluorescent dark dots in the right eye [Figure 3(A andB)]. The EDI-OCT findings included choroidalthickening, loculated spaces of the subretinal fluidwith bands, high macular retinal detachment, sub-retinal membranous structure, and hyper-reflectivedots in subretinal fluid [Figure 4 (A and B)]. Resultsof laboratory tests, including complete blood cellcount (CBC), sedimentation rate, C-reactive protein,syphilis serology, toxoplasma serology, PPD (puri-fied protein derivative) skin test, hepatitis serol-ogy, and autoimmunity markers (ANA [AntinuclearAntibody], RF [rheumatoid factor], ACE [angiotensinconverting enzyme]), and chest radiography werenegative.The initial diagnosis was VKH with unilateralclinical presentation and subclinical involvement ofthe left eye disclosed on ICGA. The patient wastreated with intravenous high-dose corticosteroid(pulse methylprednisolone 1 g/day) followed byoral prednisolone (1 mg/kg). VA and OCT find-ings significantly improved. Two months later andduring rapid tapering of the corticosteroid dosedue to systemic complications, VA of the left eyedecreased, associated with anterior chamber andvitreous cellular reaction. The notable finding wasthe presence of vitreous and anterior chamber cellsin the left eye, which was very different from thecondition of the right eye in the first presentationwith an absence of cellular reaction in anteriorchamber and vitreous. Fundoscopic examinationrevealed multiple serous retinal detachments. Slit-lamp examination and fundoscopy of the right eyehad normal results [Figure 3 (C and D), Figure 4 (Cand D)]. Therefore, the dose of prednisolone wasincreased, and methotrexate (MTX) 12.5 mg/weekwas added. VA, clinical and paraclinical exam-ination results significantly improved afterwards.The disease was in complete remission 12 monthsafter treatment with MTX and tapering of theprednisolone [Figure 4 (E and F)].DISCUSSIONVKH typically presents as a bilateral symmet-ric granulomatous uveitis. Here, we introduce acase with unilateral clinical presentation, who hadabnormalities in ICGA of both eyes. This implies the100 JOURNAL OF OPHTHALMIC AND VISION RESEARCH VOLUME 15, ISSUE 1, JANUARY-MARCH 2020A Case of Unilateral VKH Syndrome; Hosseini et alFigure 1. Fundus auto-fluorescence (FAF). (A) Hypo-autofluorescence area in macular area and hyper-auto-fluorescence ring ininferior of macula in the right eye. (B) Normal FAF in the left eye.Figure 2. Fluorescein angiography at presentation (arteriovenous phase). (A) Multifocal areas of pinpoint leakage (blue arrow),pooling (yellow arrows) staining (right eye ). (B) Normal in the left eye.importance of ICGA in the diagnosis and treatmentof VKH.VKH is diagnosed based on clinical and angio-graphic findings. Although according to the cur-rent revised diagnostic criteria, bilateral ocularinvolvement is necessary for the diagnosis of VKH,there are a few reports of unilateral or asym-metrical ocular involvement.[9, 10, 12, 13] Our patienthad clinical and paraclinical features compatiblewith VKH except for unilaterality at initial presen-tation.In this patient, except for headache, dysacu-sia, and mild hearing loss, review of systemsand laboratory tests were normal. Ophthalmicexamination, FAG, and ICGA findings were typ-ical of VKH: serous retinal detachment, focalareas of delayed choroidal perfusion, multifocalareas of pinpoint leakage, pooling, and opticnerve staining on FAG; hypofluorescent darkdots on ICGA; and choroidal thickening, locu-lated spaces of subretinal fluids with bands, highmacular retinal detachment, subretinal membra-nous structure, and hyper-reflective dots on SD-OCT. The differential diagnosis of VKH includesinfectious diseases (bacteria, fungal, tuberculosis,and syphilis), malignancies (intraocular lymphoma,diffuse uveal lymphoid hyperplasia, systemic lym-phoma, or leukemia), and inflammatory diseases(posterior scleritis, sarcoidosis, acute posteriormul-tifocal placoid pigment epitheliopathy, and multipleevanescent white dot syndrome choroidopathy).These diseases can be excluded on the basisJOURNAL OF OPHTHALMIC AND VISION RESEARCH VOLUME 15, ISSUE 1, JANUARY-MARCH 2020 101A Case of Unilateral VKH Syndrome; Hosseini et alFigure 3. Indocyanine Green angiography at presentation. (A) Early phase of the right eye reveals multiple hypo-fluorescent darkdots in posterior pole that remains hypofluorescent until late phase (blue arrow). (B) No abnormal fluorescence in early and latephase in left eye except for mild dilation of choroidal vessels (yellow arrows). (C, D) Two months after the disease presentationduring the tapering of corticosteroid: hypofluorescent dark dots in both eyes (green arrows) implying still active disease in theright eye and involvement of the left eye.of clinical manifestations, disease course, sys-temic medical workup, and ophthalmic imagingfindings.[9, 10, 12, 13]There was a significant improvement in BCVAand OCT findings in this case following theadministration of high-dose intravenous corticos-teroid. During the rapid tapering of the cor-ticosteroid due to systemic complications, fel-low eye involvement appeared clinically. Increas-ing the dose of corticosteroid and addition ofMTX caused visual acuity and fundus featuresto improve markedly. High-dose corticosteroidis the mainstay treatment of VKH. Treatmentmust be continued with gradual tapering ofthe oral corticosteroid over at least six months,along with introduction of corticosteroid-sparingagents such as MTX, mycophenolate mofetil, andcyclosporine A. Poor responders to corticosteroidsand other immunomodulatory agents would ben-efit from biological agents such as rituximab andinfliximab.[8–10, 13]In summary, VKH can present with atypicalfeatures such as unilateral or asymmetrical signsand symptoms. Meticulous diagnosis is essen-tial for prompt initiation of corticosteroids andimmunomodulatory agents, as aggressive andrapid initiation of treatment is necessary to controlthe inflammation and thereby prevent the devel-opment of sequela and visual disability. Imagingmodalities, especially ICGA, can be invaluable102 JOURNAL OF OPHTHALMIC AND VISION RESEARCH VOLUME 15, ISSUE 1, JANUARY-MARCH 2020A Case of Unilateral VKH Syndrome; Hosseini et alFigure 4. Spectral domain optical coherence tomography SD-OCT at presentation. (A) Large intraretinal cyst (red asterisk), highmacular retinal detachment (blue arrow), subretinal hyperreflective membranous structure (yellow arrow), high reflective dots(green arrow). (B) Left eye: normal. (C) Complete resolution of intraretinal cyst and subretinal hyperreflectivemembranous structure(D) but involvement of the left eye inmonth 2 during the tapering of the treatment. (E, F) Normal SD-OCT of two eyes after adequatetreatment.in the diagnosis of VKH with unilateral presen-tation, which shows subtle subclinical involve-ment.Financial Support and SponsorshipNil.Conflicts of InterestThere are no conflicts of interest.REFERENCES1. Rao NA, Gupta A, Dustin L, Chee SP, Okada AA, Khairal-lah M, et al. Frequency of distinguishing clinical fea-tures in Vogt-Koyanagi-Harada disease. Ophthalmology2010;117:591–599.e1.2. Herbort CP, Mochizuki M. Vogt–Koyanagi–Harada dis-ease: inquiry into the genesis of a disease name in thehistorical context of Switzerland and Japan. Int Ophthalmol2007;27:67–79.3. Sugita S, Takase H, Kawaguchi T, Taguchi C, MochizukiM. Cross-reaction between tyrosinase peptides andcytomegalovirus antigen by T cells from patientswith Vogt-Koyanagi-Harada disease. Int Ophthalmol2007;27:87–95.4. Rao NA. Pathology of Vogt–Koyanagi–Harada disease. IntOphthalmol 2007;27:81–85.5. Snyder DA, Tessler HH. Vogt–Koyanagi–Harada syn-drome. Am J Ophthalmol 1980;90:69–75.6. Read RW, Holland GN, Rao NA, Tabbara KF, OhnoS, Arellanes-Garcia L, et al. Revised diagnostic criteriafor Vogt-Koyanagi-Harada disease: report of an inter-national committee on nomenclature. Am J Ophthalmol2001;131:647–652.7. Lavezzo MM, Sakata VM, Morita C, Rodriguez EEC, Abdal-lah SF, da Silva FT, et al. Vogt-Koyanagi-Harada disease:review of a rare autoimmune disease targeting antigens ofmelanocytes. Orphanet J Rare Dis 2016;11:29.8. Sakata VM, Da Silva FT, Hirata CE, Marin MLC, RodriguesH, Kalil J, et al. High rate of clinical recurrence in patientswith Vogt–Koyanagi–Harada disease treated with earlyhigh-dose corticosteroids. Graef Arch Clin Exp Ophthal-mol 2015;253:785–790.9. Usui Y, Goto H, Sakai J-I, Takeuchi M, Usui M, Rao NA.Presumed Vogt-Koyanagi-Harada disease with unilateralocular involvement: report of three cases. Graef Arch ClinExp Ophthalmol 2009;247:1127–1132.10. Neves A, Cardoso A, Almeida M, Campos J, Campos A,Sousa JPC. Unilateral Vogt-Koyanagi-Harada disease: aclinical case report. Case Rep Ophthalmol 2015;6:361–365.11. Wang C, Tian Y, Lei B, Xiao X, Ye Z, Li F, et al. DecreasedIL-27 expression in association with an increased Th17response in Vogt-Koyanagi-Harada disease. Invest Oph-thalmol Vis Sci 2012;53:4668–4675.12. Cunningham ET, Jr, Rathinam SR, Tugal-Tutkun I, Muc-cioli C, Zierhut M. Vogt-koyanagi-Harada disease. OculImmunol Inflamm 2014;22:249-52.13. Forster DJ, Green RL, Rao NA. Unilateral manifestation ofthe Vogt-Koyanagi-Harada syndrome in a 7-year-old child.Am J Ophthalmol 1991;111:380–382.JOURNAL OF OPHTHALMIC AND VISION RESEARCH VOLUME 15, ISSUE 1, JANUARY-MARCH 2020 103

Vogt-Koyanagi-Harada with Unilateral Clinical Presentation: A Case Report

Abstract

Similar works

Full text

Available Versions

KnE Publishing Platform

Crossref

Neliti