Adalimumab treatment in a patient with severe presentation of VogtKoyanagi-Harada

Abstract

Context: Vogt-Koyanagi-Harada syndrome (VKH) is a rare, multisystemic, autoimmune disease mediated by a Th1 response against melanocytes in the eye, inner ear, central nervous system, skin and hair. In this article, we report a case of VKH with severe visual impairment and discuss the therapeutic response to corticotherapy followed by the use of Adalimumab, a tumor necrosis factor (TNFα) inhibitor. Case report: A 61-year-old black woman started bilateral frontal headache of severe intensity, associated with bilateral eye pain, hyperemia and watery eyes, progressing with visual turbidity with gradual worsening, seeing only figures after eight days. After ten days bilateral hypoacusis started, also progressive. She denied eye movement pain, diplopy, dizziness, fever, joint pain or skin injuries. On examination, visual acuity (VA) in RE: hand movement for 30 cm, LE: light perception, fundus of the eye with serous bilateral retinal detachment. CSF with 155 lymphomonocyte predominance cells, proteins: 73, negative bacterioscopy and cultures. Pulsotherapy was performed for 7 days followed by 1g of cyclophosphamide and maintenance therapy with fortnightly Adalimumab. Two months after discharge, she presented VA in RE: 20/200 and LE: counting fingers at 1 meter. Conclusions: Aggressive and early treatment with immunosuppression is key to the effective treatment of VKH. Immunotherapy can be used in patients who are unresponsive to corticosteroid doses. Biological agents that target TNFα have effective results in non-infectious uveitis. Adalimumab is a safe and effective option, which also reduces the need for chronic corticosteroid therapy. The prognosis depends on the early diagnosis and treatment.