Spectrum of Vogt-Koyanagi-Harada disease in North India

Purpose: To report the clinical spectrum of Vogt-Koyanagi-Harada (VKH) disease in North India. Methods: A retrospective review of VKH patients visiting the uveitis clinic of department of Ophthalmology, Post Graduate Institute of Medical Education and Research, Chandigarh, between 2003 and 2012, was done. The diagnosis of VKH disease was established by criteria from the International Committee in 2001, based on history and clinical findings with supportive evidence from ancillary tests, including ultrasound, fluorescein angiography (FA), optical coherence tomography (OCT), indocyanine green angiography (ICG), and lumbar puncture. Clinical details such as duration of uveitis, anatomic classification and course of the disease were based on the Standardization of Uveitis Nomenclature guidelines. Results: There were 120 patients (240 eyes), 40 males and 80 females. The mean age was 34.8 + 13.2 years (range 6 – 70 years). Seventy patients presented in acute phase and 50 in the chronic phase. Four patients had unilateral involvement at the time of presentation. Extraocular signs included meningismus in 40 (33.3%) patients, auditory signs in 16 (13.3%) patients and integumentary signs in 6 (5%) patients. VKH disease was diagnosed as probable in 73 (60.8%) patients, incomplete in 41 (34.2%) patients and complete in 6 (5%) patients. The median duration of symptoms was 30 days (range 3 days – 30 years). Of 240 eyes at presentation, panuveitis was seen in 137 (57.1%) eyes, keratic precipitates in 58 (24.2%) eyes (granulomatous in 7 and nongranulomatous in 51 eyes), exudative retinal detachment in 124 (51.7%) eyes, optic disc edema in 107 (44.6%) eyes, and vitreous cells in 124 (51.7%) eyes. Cataract was present in 34 (14.2%) eyes, glaucoma in 6 (2.5%), sunset glow fundus in 70 (29.2%), peripapillary atrophy in 49 (20.4%), and nummular chorioretinal scars in 53 (22.1%) eyes. Two (0.8%) eyes had subretinal fibrosis and five (2.1%) had choroidal neovascular membrane (CNVM) at presentation. 112 patients received corticosteroids (systemic 110 and local 2), and eight patients (6.7%) did not receive any treatment, as the disease was quiescent at the time of presentation. The median duration of oral corticosteroids was 20 months (range 1 month – 118 months). Seventy (58.3%) patients additionally received immunosuppressive therapy (IST). The median duration of IST was 17.5 months (range 1 month – 75 months). Of 140 eyes (70 patients) in acute phase, 44 (31.4%) eyes developed sunset glow fundus despite an initial aggressive treatment and 38 (27.1%) eyes developed peripapillary atrophy. Three eyes additionally developed CNVM on follow up. Complications included development of cataract (52 eyes, 23.2%), glaucoma (33 eyes, 14.7%), CNVM (12 eyes, 5.3%), subretinal fibrosis (4 eyes, 1.8%), cystoid macular edema (3 eyes, 1.3%), optic atrophy (glaucomatous) in 3 eyes (1.3%), ocular hypotony (3 eyes, 1.3%), macular scar (2 eyes, 0.9%), foveal atrophy (2 eyes, 0.9%), macular hole (1 eye) and band shaped keratopathy (1 eye). Conclusion: In North India, female preponderance and fourth decade were common demographic features. Extraocular signs were uncommon. Acute and chronic cases were nearly equal. Panuveitis with nongranulomatous inflammation, exudative retinal detachment, optic disc edema, sunset glow fundus and peripapillary atrophy were common initial presentations in our population. About one-third of eyes in acute phase progressed to chronic disease despite an early aggressive therapy. Eyes in chronic phase tended to develop CNVM.