Vogt-Koyanagi-Harada syndrome (VKHS) is a rare multisystemic
autoimmune disease that affects tissues containing
melanin, including the eye, inner ear, meninges, and skin. The disease is characterised by bilateral
uveitis associated with a varying constellation of auditory, neurological and cutaneous manifestations. The disease occurs more frequently among people with darker skin pigmentation. Asians, Native Americans, and Hispanics are most frequently affected. It predominates in patients aged between 20 and 50years, and females are affected more frequently, with a female:male ratio of 2:1. The classic
clinical course is characterised by bilateral
panuveitis,
hypoacusis, and
meningitis, in addition to cutaneous involvement with poliosis,
vitiligo, and
alopecia. Although the exact cause of VKH disease remains unknown, it is thought to be a T-cell-mediated autoimmune process directed against melanocytes. VKHS classically begins with vague systemic symptoms suggestive of a
viral infection, although a clear association between a specific viral agent and the disease has not been established. Genetic factors may play an important role in the loss of self-tolerance in VKHS. The
HLA-DRB1*0405 allele is the main susceptibility allele for VKHS. Early and aggressive systemic
corticosteroids are still the primary initial
therapy for VKHS. Ocular complications may require an intravitreous injection of
corticosteroids. Despite proper treatment with
steroids, a number of patients experience recurrent attacks or
steroid-associated complications. Thus, non
steroid immunomodulatory therapy (IMT) has become necessary for the treatment of VKHS.