Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes.

Marcelo Mendes Lavezzo,Viviane Mayumi Sakata,Felipe T G Da Silva,Celso Morita,Smairah Frutuoso Abdallah,Carlos Eduardo Hirata,Ever Ernesto Caso Rodriguez,Joyce Hisae Yamamoto

doi:10.1186/s13023-016-0412-4

Abstract

Vogt-Koyanagi-Harada disease (VKHD) is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. This disease is mainly a Th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of HLA-DRB1*0405 allele. The absence of ocular trauma or previous intraocular surgery sets VKHD appart from sympathetic ophthalmia, its main differential diagnosis. The disease has an acute onset of bilateral blurred vision with hyperemia preceded by flu-like symptoms. The acute uveitic stage is characterized by a diffuse choroiditis with serous retinal detachment and optic disc hyperemia and edema. Fluorescein angiography in this phase demonstrates multiple early hyperfluorescent points. After the acute uveitic stage, ocular and integumentary system pigmentary changes may appear. Ocular findings may be accompanied by lymphocytic meningitis, hearing impairment and/or tinnitus in a variable proportion of patients. Prompt diagnosis followed by early, aggressive and long-term treatment with high-dose corticosteroids is most often ensued by good visual outcomes. However, some patients may experience chronic uveal inflammation with functional eye deterioration. The current review discusses the general features of VKHD, including epidemiology, classification into categories, differential diagnosis and current therapeutic approaches.

Highlights

Vogt-Koyanagi-Harada disease (VKHD), initially described as an uveomeningoencephalitic syndrome, is a systemic granulomatous autoimmune disease that targets melanocyte-rich tissues, such as the eye, inner ear, meninges, skin and hair [1].In 1906, Alfred Vogt in Switzerland first described a patient with premature whitening of eyelashes of sudden onset and bilateral subacute iridocyclitis
Inomata and Sakamoto demonstrated a remarkable disappearance of choroidal melanocytes in VKHD eyes [29]. These findings suggested that T cell-mediated immune process against melanocytes that express class in 3a and either (II) major histocompatibility complex (MHC) play a pathogenic role in VKHD
Sympathetic ophthalmia is histopathologically identical to VKHD and can present in a similar fashion with rapid, bilateral visual loss associated with anterior segment inflammation, choroidal thickening, disc hyperemia or edema and serous retinal detachments

Summary

Introduction

Vogt-Koyanagi-Harada disease (VKHD), initially described as an uveomeningoencephalitic syndrome, is a systemic granulomatous autoimmune disease that targets melanocyte-rich tissues, such as the eye, inner ear, meninges, skin and hair [1].In 1906, Alfred Vogt in Switzerland first described a patient with premature whitening of eyelashes of sudden onset and bilateral subacute iridocyclitis. No history of ocular trauma and/or surgery; At least three of the following four signs: a) Bilateral chronic iridocyclitis; b) Posterior uveitis (multifocal exudative retinal or RPE detachments; disc hyperemia or edema; or “sunset glow fundus”, which is a yellow-orange appearance of the fundus due to depigmentation of the RPE and choroid); c) Neurologic signs (tinnitus, neck stiffness, cranial nerve or central nervous system symptoms or cerebral spinal fluid pleocytosis); d) Cutaneous findings (alopecia, poliosis or vitiligo).

Results

Conclusion