Background Vogt-Koyanagi-Harada disease is a multisystem autoimmune disorder mediated by T-cells directed against antigens of tissues that contain melanin. Clinically it's characterized by diffuse bilateral granulomatous panuveitis. Although conventional immunosuppresive therapy such as cyclosporine (CsA), azathioprine (AZA) or methotrexate (MTX) the disease can remain active and complicated by blindness.
Objectives Our aim was to review the cases of VKH syndrome uveitis in a reference unit for the past five years especially those patients with anti-TNFα therapy.
Methods We reviewed all the cases of posterior uveitis or panuveitis in a reference unit between January 2010 to December 2015. VKH syndrome was diagnosed by international standard criteria (1). The treatment was reviewed particularly those with anti-TNFa therapy refractory to conventional immunosuppresives. In addition we reviewed the literature of anti-TNF α therapy in a VKH uveitis syndrome
Results 104 were assessed. 5 cases (4 wome /1 man) met criteria VKH syndrome, mean age was 45 years. In all cases the initial treatment was high doses of corticosteroids and conventional immunosuppressive therapy CyA (4 cases), AZA (2 Cases) CsA + AZA (2 cases). In two patients with failure to AZA + CsA, infliximab (IFX) was prescribed Weeks 0, 2 and 6 and each 8 weeks. In both cases (intorelancy and ineffectiveness to infliximab), “switch” to adalimumab 40 mg/sc biweekly achieving an adequate clinical response and remission of uveitis. In addition to the two cases in our series in the literature review we found 9 articles related to anti-TNF α therapy in VKH syndrome (Table). We found 9 series with 25 patients (14 with ADA/11 IFX). In most of them remission of intraocular inflammation was observed, 13 patients with ADA (92.8%)/10 IFX (90.9%). There are no published cases of “Switching” between anti-TNF agents in VKH.
Conclusions In VKH syndrome uveitis refractory to conventional immunosuppressive, anti-TNF therapy may be a treatment option. In cases of inadequate response to the first anti-TNFa “switching” to another anti-TNFa is an option, however, these preliminary data should be tested in randomized prospective studies with larger numbers of patients.
Autoimmunity reviews. 2013; 12: 1033–1038.
Rest of references in the table (2–10),
Disclosure of Interest None declared