Vogt-Koyanagi-Harada Disease

Abstract

Vogt-Koyanagi-Harada disease (VKH) is a multisystem autoimmune disorder principally affecting pigmented tissues in the ocular, auditory, integumentary and central nervous systems. Patients are typically 20 to 50 years old and have no history of either surgical or accidental ocular trauma. Pigmented races are more commonly affected. Depending on revised diagnostic criteria, the disease is classified as complete, incomplete or probable based on the presence of extraocular findings (neurological, auditory and integumentary). The clinical course of VKH is divided into four phases: prodromal (mimics a viral infection), uveitic (bilateral diffuse uveitis with papillitis and exudative retinal detachment), convalescent (tissue depigmentation), and chronic recurrent (recurrent uveitis and ocular complications). The pathogenesis of VKH is thought to be related to an aberrant T cell-mediated immune response directed against self-antigens found on melanocytes. VKH has been linked to human leukocyte antigen DR4 (HLA-DR4) and HLA-Dw53, with strongest associated risk for HLA-DRB1*0405 haplotype. The diagnosis of VKH is clinical, and differential includes sympathetic ophthalmia, sarcoidosis, primary intraocular B-cell lymphoma, posterior scleritis, and uveal effusion syndrome. Treatment is typically initiated with high-dose oral corticosteroids, but other immunomondulatory agents (most oftentimes cyclosporine) may be needed for non-responsive patients or when corticosteroid side-effects are not tolerated. Visual prognosis is generally good with prompt diagnosis and aggressive immunomodulatory treatment.

Vogt-Koyanagi-Harada Disease

“…The precise pathogenesis remains unidentified in the vast majority of patients, however. [1][2][3][4][5][6] While corticosteroids are the mainstay of therapy and are required to control active inflammation during episodes of acute or recurrent inflammation, cumulative evidence supports the first-line use of corticosteroid-sparing immunosuppressive agents to reduce the risk of recurrence, decrease the development of late complications, and improve long-term vision. [16][17][18][19][20] Multimodal imaging studies, particularly fluorescein angiography and spectral domain-ocular coherence tomography (SD-OCT), are important adjuncts for initial diagnosis and early monitoring of treatment response.…”

“…[1][2][3][4][5][6] Four stages of VKH disease are recognized, including prodromal, acute uveitic, convalescent, and chronic/recurrent uveitic. A history of penetrating ocular trauma or surgery prior to the onset of uveitis precludes the diagnosis.…”

Recurrent Vogt–Koyanagi–Harada disease with sensorineural hearing loss and choroidal thickening

“…The extraocular symptoms are predominantly neurologic/auditory manifestations: meningismus, tinnitus, and sensorineural hearing loss [10]. Ondrey et al [11] reported that 33.3% of patients with VKH disease had sensorineural hearing loss in the acute phase despite the small sample size and that patients with untreated VKH disease might have more severe hearing abnormalities than that observed in their study, because all had already been treated for severe ocular inflammation.…”

Efusão uveal associada à tuberculose presumida: relato de caso

“…Todas as entidades clínicas causadoras de efusão cílio-coroidiana secundária formam o diagnóstico diferencial da sín-drome de efusão uveal idiopática, são elas: síndrome de VogtKoyanagi-Harada (2) ; nanoftalmia (5,7) , tumores coroidianos (metástases, linfoma, melanoma) (1,4) ; mixedema (5) ; esclerite (1) ; sífi-lis (6) ; toxoplasmose (1) ; coroidorretinopatia serosa central idiopática (12) ; fístula carótido-cavernosa (1) ; oftalmia simpática (1) e pseudotumor orbitário (1) . O presente estudo relata um caso de efusão uveal secundária, sendo a tuberculose ocular a etiologia mais provável, diagnosti- apresentações clínicas, nenhum caso evoluiu com efusão uveal (16) .…”

“…Classifica-se a efusão cílio-coroidiana ou efusão uveal como uma reação não específica observada em várias entidades clínicas (1) , podendo estar associada às doenças sistê-micas como Vogt-Koyanagi-Harada (1)(2) ; apresentar causas específicas como cirurgias oculares (3) ou pós-traumáticas (1) ; quadros não inflamatórios como leucemias (1) , mieloma múltiplo (1) , tumor de glândula lacrimal (1) , linfomas (4) , alterações vasculares (fístula carótido-cavernosa) (1) ou surgir após fotocoagulação retiniana (1) . O mixedema também é descrito como causa de efusão uveal (5) .…”