Vogt-Koyanagi-Harada综合征的治疗与预后

Vogt-Koyanagi-Harada syndrome: glucocorticoid therapy and visual prognosis

目的 观察Vogt Koyanagi Harada(VKH)综合征患者采用糖皮质激素的治疗效果 ,并探讨VKH综合征治疗的有效方案。方法 对 136例确诊为VKH综合征患者中 5 1例初诊葡萄膜炎期患者 ,采用大剂量方案治疗 ,首次泼尼松剂量为 2mg·kg-1·d-1,顿服 ,逐渐减量然后改为隔日顿服 ,疗程约为 8个月。 85例为外院转诊患者 ,均曾经糖皮质激素治疗 ,治疗时间均≥ 2个月 ,总量均≥ 2 0 0 0mg(等效剂量泼尼松 ) ;采用调整方案治疗 ,根据患者眼部情况调整泼尼松的剂量 ,疗程 6～ 10个月。同时对大剂量治疗组 11例患者及调整方案组 14例患者的 2 4h尿游离皮质醇 (urinefreecortisol,UFC)进行检测 ,以观察VKH综合征患者肾上腺皮质功能。结果 5 1例患者经大剂量方案治疗后 ,炎性反应稳定、缓解 ,94 1%的视力≥ 0 5 ,79 4 %的视力≥ 0 8,炎症复发率及眼部并发症发生率低 ;2 4hUFC水平正常。 85例经调整方案治疗后 ,炎症缓解 ,5 8 8%的视力≥ 0 5 ,4 2 9%的视力≥ 0 8为。并发症的发生率为 4 9 4 %。 2 4hUFC水平由 (5 35± 5 8) μg升至 (2 1 9± 7 2 ) μg (正常值 2 0～ 78μg/2 4h) ;结论VKH综合征患者经大剂量方案治疗效果满意 。

Objective To evaluate the efficacy of glucocorticoid therapy in patients with Vogt Koyanagi Harada (VKH) syndrome Methods One hundred and thirty six patients with VKH were treated with two regimens of oral prednisone Regimen 1: Fifty one patients with VKH were treated at the uveitic stage with prednisone, starting from 2 mg/kg/day tapering gradually and shifting to alternate day treatment The patients were treated for approximately 8 months Regimen 2: Eighty five patients with VKH were referrals who were treated elsewhere with systemic glucocorticoid more than 2 months with total dose equivalent to prednisone more than 2 000 mg Systemic and ocular complications were found in some of these patients The hypothalamus pituitary adrenal axis was markedly inhibited as indicated by decrease in urine free cortisol (UFC) These patients were treated for 6 10 months based on the patients′ individual ocular situations The visual results, frequency of recurrence of uveitis and incidence of ocular complications were compared between the two groups Results Visual acuity ≥0 5 and ≥0 8 were found to be 94 1% and 79 4% respectively in patients treated with regimen 1 which was far better than the patients treated with regimen 2 On the other hand, recurrence of uveitis and ocular complications were found significantly lower in the patients treated with regimen 1, as compared with that in patients treated with regimen 2 (23 5% vs 63 5% and 9 8% vs 49 4% respectively) All the differences are highly significant ( P <0 001) The UFC level in the patients treated with regimen 2 was increased from (5 3±5 8) μg/24 h to (21 9±7 2) μg/24 h ( P <0 001) Conclusions These results indicate that both regimens are feasible for treating patients with VKH However, regimen 1 is far better than regimen 2 with respect to visual prognosis, frequency of recurrence of uveitis as well as incidence of ocular complications