Título / Title

VOGT-KOYANAGI-HARADA SYNDROME: A RARE 13 YEAR REMISSION CASE REPORT

Introdução / Purpose

Vogt-Koyanagi-Harada Syndrome (VKHS) is a rare autoimmune disease that affects all tissues containing melanocytes such as the eyes, central nervous system, inner ear and skin. Ocular involvement is marked by bilateral, chronic granulomatous panuveitis associated with serous retinal detachment.

Material e Método / Methods

A 51-year-old female patient presented with blurred vision, ocular pain and photophobia in both eyes (OU). She was diagnosed with VKHS 13 years ago and has been in remission ever since. Best corrected visual acuity (BCVA) was 20/100 in the right eye (RE) and 20/30 in the left eye (LE). Biomicroscopy showed fine keratic precipitates in OU. Flare and anterior chamber cells were: 2+/4+ (RE) and 1+/4+ (LE). Fundoscopy showed hyperemic optic discs and serous retinal detachment (SRD) in OU. Optical coherence tomography (OCT) revealed areas of sensory RD and important diffuse thickening of the choroid in OU. Fluorescein angiography showed pin-points in OU, accumulation of contrast in the sub-retinal space in the RE and late hyperfluorescence of the optic discs in OU. A diffuse increase in permeability of choroidal vasculature was observed by green-indocyanine angiography (ICG) in OU.

Resultados / Results

The diagnosis of recurrent VKHS was given. Treatment was initiated with oral prednisone 1mg/kg/day. After 3 weeks, BCVA was 20/20 in OU with absence of active panuveitis in OU. Corticoid was slowly reduced (5mg/15 days) and azathioprine (150mg/day) was concomitantly initiated. To date, the patient remains in complete remission with decreased choroidal thickness and no signs of active disease.

Discussão e Conclusões / Conclusion

We described a rare case of recurrent VKHS after 13 years of remission. Patients with VKHS must be followed-up closely considering the risk of recurrence . ICG is the most sensitive exam to show active areas of the disease. Early treatment should be initiated with oral corticoids and then gradually replaced with immunosupressants due to corticoids long-term side effects.

Palavras Chave

Vogt-Koyanagi-Harada Syndrome; uveitis; panuveitis; serous retinal detachment; green-indocyanine angiography; immunossupression