Abstract

This retrospective study investigated the clinical characteristics and efficacy of adalimumab and low-dose methotrexate combination therapy in patients with Vogt–Koyanagi–Harada disease who were treated at Hiroshima University from February 2012 to May 2021. The patients' demographics, clinical features at administration of immunosuppressive therapy, steroid-sparing immunosuppressive therapy, side effects, and relapses were recorded. The efficacies of steroid-sparing immunosuppressive therapy (methotrexate, cyclosporine A, adalimumab, and adalimumab and methotrexate combination therapy) were analyzed. Among 62 patients, the median age at diagnosis was 47 years and the median duration of uveitis was 51 months. Systemic corticosteroid therapy was administered to 93.5% of patients (n = 58). Thirty-four patients (54.8%) were treated with steroid-sparing immunosuppressive therapy. Methotrexate and cyclosporine A were administered to 12 and 22 patients, respectively; relapse occurred in 50.0% and 22.7% of the patients, respectively. Discontinuation of cyclosporine A was required in 63.6% of patients because of side effects. Adalimumab was administered to 14 patients. Recurrence occurred in 11 patients, requiring methotrexate concomitantly. The mean dose of methotrexate at inflammatory quiescence after side effect-related dose decrease was 8.0 mg/week (0.13 mg/kg). The median duration of combination therapy without recurrence was 20 months. There were no serious adverse events during adalimumab therapy. A high relapse rate was observed in patients receiving methotrexate; a high rate of side effects requiring discontinuation was observed in patients receiving Cyclosporine A. Patients with late-stage Vogt–Koyanagi–Harada disease may achieve better control with adalimumab and methotrexate combination therapy.

Highlights

  • Vogt–Koyanagi–Harada disease (VKH) is an autoimmune bilateral panuveitis often associated with neurological and cutaneous involvement [1]

  • We focused on the clinical characteristics and treatment efficacies of MTX and cyclosporine A (CsA) in patients with VKH, as well as the use of ADA and low-dose MTX combination therapy for the treatment of late-stage VKH in Japanese patients

  • 54.8% of all patients with VKH were treated with steroid-sparing immunosuppressive therapies, including MTX, CsA, and ADA

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Summary

Introduction

Vogt–Koyanagi–Harada disease (VKH) is an autoimmune bilateral panuveitis often associated with neurological and cutaneous involvement [1]. Late-stage disease occurs in patients with insufficient treatment; this phase is characterized by depigmentation signs such as “sunset glow fundus” and recurrent uveitis, with an increased risk of ocular complications [1,2,3,4]. And aggressive systemic corticosteroid therapy is the basic treatment; systemic corticosteroid therapy alone cannot prevent the onset of late-stage disease [1, 5]. The early administration of additional steroid-sparing immunosuppressive therapy within 2–3 weeks of onset was reported to prevent the onset of late-stage VKH [6]. Vigorous early immunosuppressive therapy is recommended to prevent the onset of late-stage VKH, it is not always possible in clinical practice because of treatment availability and cost.

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