抗合成酶综合征合并间质性肺疾病患者的临床特点及初始治疗效果分析  

Analysis of Clinical Characteristics and Initial Treatment Outcomes of Patients with Antisynthetase Syndrome With Interstitial Lung Disease

作　　者：彭岩 卢立国 张毛为 孙宜田 刘亚男 陈碧 PENG Yan;LU Liguo;ZHANG Maowei;SUN Yitian;LIU Yanan;CHEN Bi(Department of Respiratory and Critical Care Medicine,Shuyang Hospital,Suqian,Jiangsu 223600,China;The Affiliated Hospital of Xuzhou University,Xuzhou,Jiangsu 221004,China;Department of Respiratory and Critical Care Medicine,Affiliated Hospital of Xuzhou University,Xuzhou,Jiangsu 221000,China)

机构地区：[1]沭阳医院呼吸与危重症医学科,江苏宿迁223600 [2]徐州医科大学,江苏徐州221004 [3]徐州医科大学附属医院呼吸与危重症医学科,江苏徐州221000

出　　处：《临床肺科杂志》2025年第3期337-342,共6页Journal of Clinical Pulmonary Medicine

基　　金：江苏省卫健委医学科研面上项目(H2023005);江苏省高校重点实验室开放课题(XZSYSKF2022004)。

摘　　要：目的总结首诊于呼吸科的抗合成酶综合征合并间质性肺疾病(antisynthetase syndrome with interstitial lung disease,ASS-ILD)患者的临床特点,并探讨初始治疗效果的影响因素。方法回顾性分析了2019年9月至2023年5月在徐州医科大学附属医院呼吸与危重症学科首诊的59例ASS患者的临床资料。根据初始治疗效果将患者分为治疗反应良好组和治疗反应不良组,并采用Logistic多因素回归分析了初始治疗效果的影响因素。结果59例患者中,女性36例(61.0%),年龄范围32~83岁,中位年龄64(56,70)岁。主要表现为咳喘,肌无力、技工手等其他临床特点不突出。抗合成酶抗体中以抗Jo-1抗体阳性率最高。肺功能多呈限制性通气功能障碍和换气功能障碍。胸部影像学表现以非特异性间质性肺炎为主,其次是机化性肺炎。治疗上单用糖皮质激素或联合免疫抑制剂,初始治疗效果良好者占45.8%。Logistic多因素回归显示抗Jo-1抗体阳性(OR=9.895%CI 1.03~83.04,P=0.04)、免疫抑制剂应用(OR=20.8995%CI 1.39~56.31,P=0.03)和影像学分型含OP(即机化性肺炎型,或非特异性间质性肺炎合并机化性肺炎型)(OR=13.7795%CI 2.06~96.92,P=0.01)是初始治疗效果良好的促进因素。结论首诊于呼吸科的抗合成酶综合征患者以间质性肺病为主要表现,糖皮质激素联合免疫抑制剂对初始治疗效果有益,胸部影像学表现及分型对初始治疗有预测作用。Objective To summarize the clinical characteristics of patients with antisynthetase syndrome with interstitial lung disease(ASS-ILD)initially diagnosed in the respiratory department,and to explore the influencing factors of initial treatment.Methods A retrospective analysis was conducted on the clinical data of 59 patients with ASS who were initially diagnosed in the Department of Respiratory and Critical Care Medicine at Affiliated Hospital of Xuzhou Medical University from September 2019 to May 2023.The patients were divided into the good treatment response group and the poor treatment response group based on the initial treatment effect,and Logistic multivariate regression analysis was used to analyze the influencing factors of the initial treatment effect.Results Among the 59 patients,there were 36 females(61.0%)with an age range of 32-83 years and a median age of 64(56,70)years.The main symptoms were cough and wheezing,with less prominent features such as muscle weakness and mechanic′s hands.The positive rate of the anti-Jo-1 antibody was the highest among the antisynthetase antibodies.Pulmonary function mainly showed restrictive ventilatory dysfunction and impaired gas exchange.The main chest CT findings were nonspecific interstitial pneumonia(NSIP),followed by organizing pneumonia(OP).Treatment mainly consisted of corticosteroids alone or in combination with immunosuppressants,and 45.8%of patients showed a good initial treatment response.Logistic multivariate regression analysis showed that positive anti-Jo-1 antibody(OR=9.895%CI 1.03-83.04,P=0.04),immunosuppressant use(OR=20.8995%CI 1.39-56.31,P=0.03),and imaging types including OP(organizing pneumonia,or nonspecific interstitial pneumonia combined with organizing pneumonia)(OR=13.7795%CI 2.06-96.92,P=0.01)were promoting factors for good initial treatment response.Conclusion Patients with ASS initially diagnosed in the respiratory department mainly present with interstitial lung disease,and corticosteroids combined with immunosuppressants are beneficial

关 键 词：间质性肺病 抗合成酶综合征 胸部高分辨CT 免疫抑制剂 初始治疗反应 

分 类 号：R593.2[医药卫生—内科学] R563[医药卫生—临床医学]