重组人干扰素α2a治疗难治性白塞综合征的疗效及安全性

Efficacy and safety of recombinant human interferon alpha 2a in refractory Beh et syndrome

目的探讨重组人干扰素α2a(interferonα2a,IFN-α2a)治疗难治性白塞综合征(Beh et syndrome,BS)的疗效和安全性。方法回顾性分析北京协和医院2014年11月至2019年7月收治的9例BS患者因顽固性黏膜溃疡、并血管或神经系统受累,接受IFN-α2a治疗的临床资料。结果9例患者(包括7例男性和2例女性)平均年龄(36.55±5.03)岁,病程(122±84.45)个月。其中顽固性黏膜溃疡3例,反复多发静脉血栓1例,多发下肢动脉狭窄、闭塞,下肢深静脉血栓伴发血栓后综合征1例,神经系统受累3例(1例并发顽固黏膜溃疡),另1例同时并神经、血管、眼受累,经激素联合免疫抑制剂积极治疗后临床无明显改善,且4例并发乙肝病毒感染,1例并发肺结核。接受IFN-α2a治疗后,平均随访(9.78±2.72)个月,所有患者临床症状均有改善,皮质类固醇激素使用剂量减少,随访6个月时中位泼尼松剂量从10(0,60)mg逐渐减少到5(0,15)mg,末次随访中位泼尼松剂量为4.375(0,12.5)mg(P<0.05);中位免疫抑制剂种类从2(1,3)种逐渐减少至1(0,2)种,末次随访中位免疫抑制剂种类为1(0,1)种(P<0.05)。1例(12.5%)患者停用激素,3例(33.3%)患者停用免疫抑制剂。结论IFN-α2a与皮质类固醇激素和免疫抑制剂联合使用,对治疗难治性BS有效且耐受性良好,有利于皮质类固醇激素和免疫抑制剂减量。

Objective To investigate the effectiveness and safety of human recombinant interferon alpha 2a in refractory Beh et syndrome(BS).Methods In this retrospective cohort study,nine patients with refractory BS were included.They had intractable mucosal ulcer,vascular or neurological involvement,and received IFN alpha 2a treatment in Peking Union Medical College Hospital between November 2014 and July 2019.Results A total of 9 patients(7 males and 2 females)were included,with a median age of(36.55±5.03)years and a disease duration of(122±84.45)months.Among these patients,three had an intractable mucosal ulcer,one patient had recurrent deep vein thrombosis,one had multiple arterial stenosis and occlusion,with deep vein thrombosis and post-thrombotic syndrome.Three patients had neurological involvement,and one of them had intractable mucosal ulcer concurrently.One patient with vascular,neurological and ocular involvement deteriorated on corticosteroid and immunosuppressants.Four patients complicated with hepatitis B virus(HBV)infection and one with active tuberculosis.The median follow-up time was(9.78±2.72)months on interferon-alpha 2a.All patients reported improvement of symptoms.The median dose of prednisone tapered from 10(0,60)mg to 5(0,15)mg after six months of follow-up and the median types of immunosuppressants reduced from 2(1,3)to 1(0,2).Prednisone dosage and the types of immunosuppressants was reduced significantly on last visit,which was 4.375(0,12.5)mg(P<0.05)and 1(0,1)(P<0.05),respectively.One patient(12.5%)stopped prednisone and three patients(33.3%)stopped immunosuppressants at last.Conclusion Interferon-alpha 2a in combination with corticosteroid and immunosuppressants is effective and well-tolerated in the treatment of refractory BS and benefitted corticosteroid and immunosuppressants tapering.