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Vogt-Koyanagi-Harada disease

2002; Elsevier BV; Volume: 15; Issue: 3 Linguagem: Inglês

10.1016/s0896-1549(02)00025-1

1558-3856

Russell W. Read,

Inflammasome and immune disorders

Vogt-Koyanagi-Harada (VKH) disease has been known, but not necessarily recognized in its complete form, for over a millennium. Despite this, information regarding the exact cause and best treatment regimen for this potentially blinding condition are not established. Manifesting ophthalmologically as a bilateral, granulomatous panuveitis, VKH disease seems to be a T-cell mediated systemic autoimmune process, which evidence increasingly suggests is directed at one or more antigens associated with melanocytes. As such, any organ system that contains melanocytes is a potential target of attack, including the eye, skin and hair, inner ear, and meninges. VKH disease occurs more frequently in certain ethnic groups. The disease has distinct phases, with acute disease involving primarily the ocular posterior segment, central nervous system, and inner ear. By contrast, chronic-recurrent disease involves the ocular anterior segment and integumentary system. Aggressive anti-inflammatory therapy is typically required, but no quality clinical trial data exist to guide the agent of choice, dosage, or duration of therapy. Complications are not uncommon and are typically the reason for decreased vision.