北京大学学报(医学版)2024,Vol.56Issue(2) :284-292.DOI:10.19723/j.issn.1671-167X.2024.02.013

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

赖展鸿 李嘉辰 贠泽霖 张永刚 张昊 邢晓燕 邵苗 金月波 王乃迪 李依敏 李玉慧 栗占国
北京大学学报(医学版)2024,Vol.56Issue(2) :284-292.DOI:10.19723/j.issn.1671-167X.2024.02.013

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

赖展鸿 1李嘉辰 1贠泽霖 1张永刚 2张昊 3邢晓燕 1邵苗 1金月波 1王乃迪 1李依敏 4李玉慧 1栗占国1
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作者信息

  • 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
  • 折叠

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 intersti-tial 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 immuno-globin,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.

Abstract

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 in-cluded.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 syn-drome(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 re-sponse 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 treat-ment,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.Conclu-sion:The total complete clinical response rate of IIMs is low,especially for IMNM subtype.More effec-tive intervention should be administered to patients with ILD,elevated AST,elevated serum ferritin or decreased count of CD4+T cells at disease onset.

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

Key words

Idiopathic inflammatory myopathies/Clinical response/Interstitial lung disease/Risk fac-tors/Autoantibodies

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出版年

2024
北京大学学报(医学版)
北京大学

北京大学学报(医学版)

CSTPCDCSCD北大核心
影响因子:1.681
ISSN:1671-167X
参考文献量45
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