托法替布治疗MDA5抗体阳性皮肌炎合并间质性肺病7例并文献复习

Application of tofacitinib in treatment of seven patients with anti-MDA5 autoantibody-positive dermatomyositis-associated with interstitial lung disease and literature review

目的报道7例经托法替布治疗的抗黑色素瘤分化相关基因蛋白5(MDA5)抗体阳性皮肌炎合并间质性肺病(ILD)患者的临床特点、诊治经过及预后并进行文献复习,以提高对该病的认识。方法收集解放军总医院第一医学中心风湿免疫科2018年1月-2021年10月收治的7例MDA5抗体阳性皮肌炎合并ILD患者,回顾性分析其应用托法替布治疗前后的临床症状、实验室检查指标、影像学表现变化及不良反应发生情况。检索相关数据库,结合文献报道的36例患者,总结托法替布治疗MDA5抗体阳性皮肌炎合并ILD的有效性及安全性。结果7例患者中5例为女性,2例为男性;年龄50(30~64)岁,病程3(1~36)个月,随访时间12(1~35)个月。7例中有4例既往曾接受其他免疫抑制剂治疗,3例为初治患者。1例死亡,6例病情稳定,维持治疗。1例患者在使用托法替布后的第18个月出现真菌感染。国内外报道托法替布治疗MDA5抗体阳性皮肌炎合并ILD患者共36例,治疗后临床症状、实验室检查指标及影像学表现均有改善,应用托法替布后9例(25%)发生机会性感染。结论托法替布治疗MDA5抗体阳性皮肌炎合并ILD的疗效显著,但在治疗期间应注意监测机会性感染。

Objective By literature review to retrospectively study the clinical characteristics,diagnosis and treatment process,and prognosis of anti-melanoma differentiation associated gene 5(MDA5)autoantibody-positive dermatomyositisassociated with interstitial lung disease(ILD)treated with tofacitinib(TOF),so as to improve the understanding of the disease.Methods Retrospectively analyze the clinical and laboratory characteristics,imaging findings and adverse reactions before and after TOF treatment in seven patients who admitted to the First Medical Center of Chinese PLA General Hospital from January 2018 to October 2021.Thirty-six cases were found by searching databases to summarize the efficacy and safety of TOF in treating patients with anti-MDA5 autoantibody-positive dermatomyositis-associated ILD.Results Of the 7 patients,5 were female and 2 were male with mean age of 50(30-64)years,the mean disease duration was 3(1-36)months,and the mean follow-up time was 12(1-35)months.Of them,4 received previously the other immunosuppressive therapy,and 3 were newly treated.One patient died,and the remaining 6 patients were in relatively stable condition and maintenance treatment.One patient experienced fungal infection in the 18th month after TOF treatment.A total of 36 cases of anti-MDA5 autoantibody-positive dermatomyositis have been reported in databases.Clinical symptoms,laboratory tests and imaging findings were all improved after TOF therapy.Opportunistic infection occurred in 9(25%)cases after application of TOF.Conclusion Application of TOF is an effective and safe regimen in treatment of anti-MDA5 autoantibody-positive dermatomyositis-associated ILD,while attention should be paid for monitoring the opportunistic infection during treatment.