High rate of clinical recurrence in patients with Vogt–Koyanagi–Harada disease treated with early high-dose corticosteroids

Purpose To analyse the rate of clinical recurrences in Brazilian patients with Vogt–Koyanagi–Harada (VKH) disease after early high-dose corticosteroid treatment. Methods Retrospective study including patients treated with early high-dose corticosteroids (prednisone, 1–1.5 mg/kg/day, or 3-day 1 g methylprednisolone pulsetherapy) within 1 month from disease onset followed by slow taper (at least 6 months). Patients with a minimum 12-month follow-up were subdivided based on the presence of disease recurrence or persistence after 6 months from initial presentation into: acute–resolved (AR, no recurrences), chronic–recurrent (CR), and chronic–recurrent with subretinal fibrosis (SRF). Recurrences were defined as the presence of clinical and/or fluorescein angiography findings. Results Twenty-nine patients (58 eyes) with a median follow-up of 65 months were included. Six (21 %), 11 (38 %) and 12 (41 %) patients were allocated to AR, CR, and SRF groups respectively. Though having received treatment within 1 month of onset, median time to initial treatment differed among groups (11, 15, and 25 days, in AR, CR, and SRF groups respectively). Intensity of immunosuppression, cataract development, and longer time to achieve logMAR visual acuity ≤0.8 differed significantly among the groups, being more severe in SRF group. HLA-DRB1*0405 allele followed the same trend, though not reaching significance (0.5 in AR group, 0.6 in CR, and 0.8 in SRF). Conclusion VKH disease in Brazilian patients evolved to chronic–recurrent disease in 79 % of cases; 38 % developed subretinal fibrosis, in spite of similar initial treatment regimens. Time to initiate treatment influenced outcomes.