FRI0482 Anti-TNFα Therapy and Switching in Severe Uveitis of Vogt-Koyanagi-Harada Syndrome. Report of Two Cases and Literature Review: Table 1

Abstract

BackgroundVogt-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.ObjectivesOur 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.MethodsWe 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 syndromeResults104 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.Table 1Present series. Flores Robles et al.Martínez et al.Díaz-Lagares et al.Zmuda et al.Khan et al.Díaz-Llopis et al.Nicolli et al.Jeroudis et al.Díaz-Llopis et al.Yosuf et al.SpainSpain (2)Spain (3)France (4)USA (5)Spain (6)Italy (7)USA (8)Spain (9)USA (10)Number of Cases2342212182Age (mean in years)43.540Not specified (NS)35.514.53639.515NS11Previous immunosuppressive or biologicCYA and AZAMTX (2 cases)CyS (4 cases)MTXMTX (1 case)CySAZA (2 cases)MTXNSMTX (2 cases)CyS (3 cases)MTX (2 cases)CyS (1 case)CyS (2 cases)MMF (1 case)AZA (1 case)Tacrolimus (1 case)CyS (1 case)AZA (1 case)MMF (1 case)Biologic usedIFX (n=2), ADA (n=2)IFXADAIFXIFXADAIFXADAIFXIFXReason for initiating anti-TNFαImmunosuppressant failure (IF)IFIFFirst-lineIFCyS failureIFMTXfailureIFFirst-linePatients with clinical response2332212181Adverse effects due to anti-TNFα1NoneNone(1 case)NoneNoneNoneNoneNot specifiedNoneDuration of response18 monthsNot specified9.5 months7 months17 months8 months27 months26 monthsNS12 months (1 patient)ConclusionsIn VKH syndrome uveitis refractory to conventional immunosuppressive, anti-TNF therapy may be a treatment option. In cases of inadequate response to the ...