Atypical case of Vogt- Koyanagi-Harada disease

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Presentation transcript:

Atypical case of Vogt- Koyanagi-Harada disease Rim Kahloun Nesrine Abroug Moncef Khairallah Department of ophthalmology Fattouma Bourguiba University Hospital Faculty of Medicine,University of Monastir Monastir, Tunisia

History 53-year-old woman History of systemic hypertension Progressive decrease in visual acuity in the left eye (OS)

First Presentation Visual acuity: 20/32 OD; 20/100 OS No cells or flare in anterior chamber OD 0.5+ anterior chamber cells OS Intraocular pressure: 19 mmHg OD, 25 mmHg OS No cells or flare in vitreous OU Anterior segment photograph was normal OD, and shows a sunset glow ‘pupil’ appearance and posterior synechiae OS.

Fundus examination shows optic disc edema and multifocal serous retinal detachment OD, and a sunset glow fundus OS.

Fluorescein angiography B C Early (A) and mid-phase (B) fluorescein angiogram OD show areas of delayed choriocapillaris filling. (C) Late-phase fluorescein angiogram shows multiple pinpoints and optic disc staining.

Fluorescein angiography B Early-phase (A) and late-phase (B) fluorescein angiogram OS show areas of retinal pigment epithelial changes predominating around optic disc

Optical coherence tomography OD: OCT shows ERD with multifocal folds of the retinal pigment epithelium and Bruch’s membrane OS: normal

Ultrasonography A B OD (A): 20-MHz ultrasonography shows diffuse low- to medium-reflective choroidal thickening most marked in the posterior fundus OS (B): choroidal atrophy

Initial differential diagnosis Atypical Vogt-Koyanagi-Harada disease Sympathetic Ophthalmia Posterior scleritis

Work-up Internal Medicine examination: normal Syphilis serology: negative

Atypical Vogt Koyanagi Harada (VKH) disease Final diagnosis Atypical Vogt Koyanagi Harada (VKH) disease

Treatment Systemic prednisone 1 mg/Kg/day

Follow-up Three months after Visual acuity: 20/25 OD; 20/32 OS Sunset glow fundus Normal fundus appearance

Conclusion Atypical case of VKH disease with features of acute phase in one eye and chronic convalescent phase in the fellow eye It may be related to an asymmetric T cell-mediated autoimmune reaction against one or more antigens found on or associated with melanocytes Awareness of asymmetric or “apparently” unilateral acute VKH disease is of utmost importance for early initiation of corticosteroid therapy to prevent chronic disease, recurrences, complications, and subsequent visual loss