中国全科医学 ›› 2024, Vol. 27 ›› Issue (13): 1623-1629.DOI: 10.12114/j.issn.1007-9572.2022.0003

• 论著 • 上一篇    下一篇

特发性炎性肌病患者临床特点及发生肺间质病变的危险因素研究

李兴珺1,2, 李双蓉1,3, 王楠1, 王湘宇1, 崔若玫1, 徐健1, 郭雨龙4, 刘爽1,*()   

  1. 1.650032 云南省昆明市,昆明医科大学第一附属医院风湿免疫科
    2.625000 四川省雅安市,雅安职业技术学院
    3.643000 四川省自贡市第四人民医院
    4.650000 云南省昆明市,云南省阜外心血管病医院
  • 收稿日期:2023-09-07 修回日期:2023-12-28 出版日期:2024-05-05 发布日期:2024-03-06
  • 通讯作者: 刘爽

  • 作者贡献:

    李兴珺负责数据统计及论文初步撰写;李双蓉、王楠参与数据收集;王湘宇、崔若玫及徐健参与论文后期修改;郭雨龙负责统计分析及指导;刘爽负责整篇文章修改及统计分析,对文章整体负责。

  • 基金资助:
    国家自然科学基金资助项目(32270947); 云南省科技厅-昆明医科大学应用基础研究联合基金(202301AY070001-043,202301AY070001-159); 云南省"万人计划"青年拔尖人才(YNWR-QNBJ-2018-152); 云南省"万人计划"名医(YN-WR-MY-2018-040); 昆明医科大学第一附属医院"535"高层次人才青年学术骨干(2022535Q01); 云南省高层次卫生技术人才领军人才(L-2019004); 白求恩普爱医学研究基金(PAYJ-043); 云南省皮肤免疫性疾病临床医学中心(ZX2019-03-02)

Clinical Characteristics of Patients with Idiopathic Inflammatory Myopathy and Risk Factors for the Development of Interstitial Lung Disease

LI Xingjun1,2, LI Shuangrong1,3, WANG Nan1, WANG Xiangyu1, CUI Ruomei1, XU Jian1, GUO Yulong4, LIU Shuang1,*()   

  1. 1. Department of Rheumatology and Immunology, First Affiliated Hospital of Kunming Medical University, Kunming 650032, China
    2. Ya'an Polytechnic College, Yaan 625000, China
    3. Fourth People's Hospital of Zigong City, Zigong 643000, China
    4. Yunnan Fuwai Cardiovascular Hospital, Kunming 650000, China
  • Received:2023-09-07 Revised:2023-12-28 Published:2024-05-05 Online:2024-03-06
  • Contact: LIU Shuang

摘要: 背景 特发性炎性肌病(IIM)是一组以肌肉炎症和肌无力为主要特征的结缔组织病,肺部受累是影响患者预后的重要因素。根据肌炎特异性抗体(MSAs),可将IIM分为不同的临床亚型,不同亚型的IIM患者其临床表现、器官受累和预后均有所不同,发生肺间质病变(ILD)的风险也不相同。 目的 探讨IIM及其不同临床亚型的特点与其发生ILD的危险因素。 方法 收集并整理2018年4月—2021年2月于昆明医科大学第一附属医院风湿免疫科住院并诊断为IIM患者的临床资料,基于MSAs,将其分为抗MDA5抗体阳性皮肌炎(DM)、抗MDA5抗体阴性皮肌炎、坏死性肌炎(IMNM)和抗合成酶综合征(ASS)4个临床亚型,比较不同亚型患者的一般资料、临床表现、实验室检查结果,建立多因素Logistic回归模型,探索IIM患者发生ILD的危险因素。 结果 将150例IIM患者分为4个临床亚型,其中抗MDA5抗体阳性DM患者30例(20.0%),抗MDA5抗体阴性DM患者58例(38.7%),IMNM患者14例(9.3%),ASS患者48例(32.0%)。不同亚型患者的肌无力、肌痛、ILD、向阳疹、披肩征、Gottron疹/征、关节痛、甲周红斑及吞咽困难的发生率比较,差异均有统计学意义(P<0.05);其中抗MDA5抗体阳性DM亚型和ASS亚型患者的ILD发生率均高于抗MDA5抗体阴性DM亚型和IMNM型(P<0.05),抗MDA5抗体阳性或阴性DM亚型患者的向阳疹、披肩征发生率均高于IMNM亚型和ASS亚型(P<0.05),抗MDA5抗体阳性DM患者关节痛发生率高于抗MDA5抗体阴性DM患者(P<0.05)。不同临床亚型患者的白细胞计数(WBC)、丙氨酸氨基转移酶(ALT)、天门冬氨酸氨基转移酶(AST)、血肌酐、乳酸脱氢酶(LDH)、肌酸激酶(CK)、C4、铁蛋白、T淋巴细胞、CD8+T淋巴细胞及自然杀伤细胞(NK细胞)比较,差异均有统计学意义(P<0.05)。不同临床亚型的IIM患者,ILD发生率比较,差异有统计学意义(P<0.05)。多因素Logistic回归分析发现抗MDA5抗体阳性、抗合成酶抗体阳性、肺部感染、铁蛋白>403.2 μg/L、IgG>14.15 g/L、LDH>359.5 U/L均是IIM发生ILD的危险因素(P<0.05)。 结论 不同临床亚型患者的临床表现有明显差异,抗MDA5抗体阳性的DM患者更易有皮疹、关节痛、ILD、WBC减少等临床表现。MDA5抗体阳性、抗合成酶抗体阳性、肺部感染、铁蛋白、LDH及IgG升高是IIM发生ILD的危险因素。

关键词: 特发性炎性肌病, 临床亚型, 肌炎特异性抗体, 肺间质病变, 危险因素

Abstract:

Background

Idiopathic inflammatory myopathy (IIM) is a group of connective tissue diseases characterized by muscle inflammation and muscle weakness, lung involvement is an important factor affecting the prognosis of patients. IIM can be classified into different clinical subtypes based on myositis-specific antibodies (MSAs), there are significant differences in clinical manifestations, organ involvement, prognosis and the risk of interstitial lung disease among different clinical subtypes.

Objective

To explore the characteristics of IIM and its different clinical subtypes, the risk factors for the development of interstitial lung disease.

Methods

The clinical data of patients hospitalizedin department of rheumatology and immunology of the First Affiliated Hospital of Kunming Medical University and diagnosed with IIM from April 2018 to February 2021 were collected. The included patients were divided into four clinical subtypes based on MSAs, including anti-MDA5-positive dermatomyositis (DM), anti-MDA5-negative DM, immune-mediated necrotizing myositis (IMNM) and anti-synthetase syndrome (ASS) subtypes. The general data, clinical manifestations, laboratory examination results were compared among different clinical subtypes. Multivariate Logistic regression model was established to explore the risk factors for ILD in patients with IIM.

Results

The 150 patients with IIM were divided into 4 clinical subtypes, including 30 patients with anti-MDA5-positive DM subtype (20.0%), 58 patients with anti-MDA5-negative DM subtype (38.7%), 14 patients with IMNM subtype (9.3%), and 48 patients with ASS subtype (32.0%). There were significant differences in the incidence of muscle weakness, myalgia, ILD, heliotrope rash, shawl sign, Gottron papules or Gottron sign, arthralgia, periungual red spot and dysphagia among the four clinical subtypes (P<0.05). The incidence of ILD in patients with anti-MDA5-positive DM and ASS subtypes was higher than patients with anti-MDA5-negative DM and IMNM subtypes, respectively (P<0.05) ; The incidences of heliotrope rash and shawl sign in patients with anti-MDA5-positive DM and anti-MDA5-negative DM subtypes were higher than patients with IMNM and ASS subtypes (P<0.05) ; The incidences of arthralgia in patients with anti-MDA5-positive DM subtype was higher than patients with anti-MDA5-negative DM subtype (P<0.05). There were significant differences in the levels of WBC, ALT, AST, serum creatinine, LDH, CK, C4, ferritin, T cell, CD8+ T cell, nature kill (NK) cell and incidence of ILD among patients with different subtypes (P<0.05). Multivariate Logistic regression analysis showed that anti-MDA5 antibody positive, anti-synthetase antibody positive, lung infection, ferritin>403.2 μg/L, IgG>14.15 g/L, LDH>359.5 U/L were all risk factors for ILD in IIM (P<0.05) .

Conclusion

The clinical manifestations of patients with different clinical subtypes differ significantly. DM patients with anti-MDA5 antibody positive are more likely to develop rash, arthralgia, ILD and leukopenia. MDA5 antibody positive, anti-synthetase antibody positive, lung infection and elevated ferritin, LDH and IgG levels are the risk factors for IIM with ILD.

Key words: Idiopathic inflammatory myopathy, Clinical subtypes, Myositis-specific antibodies, Interstitial lung disease, Risk factors

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