特发性炎性肌病完全临床应答相关因素的单中心真实世界研究  

A unicenter real-world study of the correlation factors for complete clinical response in idiopathic inflammatory myopathies

作　　者：赖展鸿 李嘉辰 贠泽霖 张永刚 张昊[3] 邢晓燕 邵苗[1] 金月波[1] 王乃迪 李依敏 李玉慧[1] 栗占国[1] LAI Zhanhong;LI Jiachen;YUN Zelin;ZHANG Yonggang;ZHANG Hao;XING Xiaoyan;SHAO Miao;JIN Yuebo;WANG Naidi;LI Yimin;LI Yuhui;LI Zhanguo(Department of Rheumatology and Immunology,Peking University People’s Hospital,Beijing 100044,China;Department of Rheumatology and Immunology,Baoding First Hospital,Baoding 071000,Hebei,China;Department of Rheumatology and Immunology,Dalian Municipal Central Hospital,Dalian 116089,Liaoning,China;Department of Rheumatology and Immunology,The First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China)

机构地区：[1]北京大学人民医院风湿免疫科,北京100044 [2]保定市第一中心医院风湿免疫科,河北保定071000 [3]大连市中心医院风湿免疫科,辽宁大连116089 [4]浙江大学医学院附属第一医院风湿免疫科,杭州310003

出　　处：《北京大学学报（医学版）》2024年第2期284-292,共9页Journal of Peking University:Health Sciences

摘　　要：目的:探究影响特发性炎性肌病(idiopathic inflammatory myopathies, IIMs)患者对常规治疗完全临床应答的相关因素。方法:纳入2000年1月至2023年6月于北京大学人民医院就诊的IIMs患者,通过分析患者的临床特征、实验室检查、免疫学指标和治疗用药,明确影响患者对常规治疗完全临床应答的相关因素。结果:共纳入635例IIMs患者,其中518例患者完成随访,平均随访时间36.8个月,总体完全临床应答率为50.0%(259/518)。各临床亚型中,皮肌炎(dermatomyositis, DM)、抗合成酶综合征(anti-synthetase syndrome, ASS)和免疫介导坏死性肌病(immune-mediated necrotizing myopathy, IMNM)的完全临床应答率分别为53.5%、48.9%和39.0%。未完全临床应答组与完全临床应答组相比,在临床特征方面,发热(P=0.002)和快速进展型间质性肺病(rapid progressive interstitial lung disease, RP-ILD)(P=0.014)的发生率较高;在实验室检查方面,谷草转氨酶、乳酸脱氢酶、D-二聚体、红细胞沉降率、C反应蛋白和血清铁蛋白水平较高;在治疗用药方面,激素和静脉注射免疫球蛋白(intravenous immunoglobin, IVIG)的使用比例均较高。IMNM(P=0.007)、间质性肺病(P=0.001)、谷草转氨酶高(P=0.012)、血清铁蛋白高(P=0.016)和外周血CD4+T细胞计数低(P=0.004)是IIMs未完全临床应答的危险因素。结论:IIMs患者的总体完全临床应答率低,IMNM亚型最低;起病时存在间质性肺病、谷草转氨酶高、血清铁蛋白高或外周血CD4+T细胞计数低的患者应给予积极治疗。Objective:To investigate the correlation factors of complete clinical response in idiopathic inflammatory myopathies(IIMs)patients receiving conventional treatment.Methods:Patients diagnosed with IIMs hospitalized in Peking University People’s Hospital from January 2000 to June 2023 were included.The correlation factors of complete clinical response to conventional treatment were identified by analyzing the clinical characteristics,laboratory features,peripheral blood lymphocytes,immunological indicators,and therapeutic drugs.Results:Among the 635 patients included,518 patients finished the follow-up,with an average time of 36.8 months.The total complete clinical response rate of IIMs was 50.0%(259/518).The complete clinical response rate of dermatomyositis(DM),anti-synthetase syndrome(ASS)and immune-mediated necrotizing myopathy(IMNM)were 53.5%,48.9%and 39.0%,respectively.Fever(P=0.002)and rapid progressive interstitial lung disease(RP-ILD)(P=0.014)were observed much more frequently in non-complete clinical response group than in complete clinical response group.The aspartate transaminase(AST),lactate dehydrogenase(LDH),D-dimer,erythrocyte sedimentation rate(ESR),C-reaction protein(CRP)and serum ferritin were significantly higher in non-complete clinical response group as compared with complete clinical response group.As for the treatment,the percentage of glucocorticoid received and intravenous immunoglobin(IVIG)were significantly higher in non-complete clinical response group than in complete clinical response group.Risk factor analysis showed that IMNM subtype(P=0.007),interstitial lung disease(ILD)(P=0.001),eleva-ted AST(P=0.012),elevated serum ferritin(P=0.016)and decreased count of CD4+T cells in peripheral blood(P=0.004)might be the risk factors for IIMs non-complete clinical response.Conclusion:The total complete clinical response rate of IIMs is low,especially for IMNM subtype.More effective intervention should be administered to patients with ILD,elevated AST,elevated serum ferritin or decreased coun

关 键 词：特发性炎性肌病 临床应答 间质性肺病 危险因素 自身抗体 

分 类 号：R593.26[医药卫生—内科学]