非抗Jo-1抗体阳性特发性炎性肌病患者的临床特点和预后分析  

Clinical Characteristics and Prognosis of Patients with Non-anti-Jo-1 Antibody Positive Idiopathic Inflammatory Myopathy

作　　者：杨露伟 戴晓敏[2] 丁振奇 於强[2] 陈慧勇[2] 马莉莉[2] 姜林娣[2] YANG Luwei;DAI Xiaomin;DING Zhenqi;YU Qiang;CHEN Huiyong;MA Lili;JIANG Lindi(Xiamen Branch of Zhongshan Hospital of Fudan University,Xiamen 361000,China)

机构地区：[1]复旦大学附属中山医院厦门医院,福建厦门361000 [2]复旦大学附属中山医院

出　　处：《中国医学创新》2022年第33期1-6,共6页Medical Innovation of China

基　　金：国家自然科学基金资助项目(81901639)。

摘　　要：目的:分析及总结非抗Jo-1抗合成酶抗体阳性的特发性炎性肌病患者的临床特点及预后情况。方法:回顾性收集2011年1月-2021年6月复旦大学附属中山医院风湿科住院且进行抗合成酶抗体检测阳性的特发性炎性肌病患者的一般资料、实验室检查、疾病表现和肺部影像学资料,观察期到患者死亡或2022年6月1日,根据患者抗合成酶抗体类型分为抗Jo-1阳性与抗Jo-1阴性患者,比较两者临床特点及预后情况。结果:共纳入125例抗合成酶抗体阳性的患者,抗Jo-1抗体、抗PL-7抗体、抗PL-12抗体、抗OJ抗体及抗EJ抗体阳性患者分别有70、17、6、3、29例,抗Jo-1抗体阳性患者关节炎、肌无力的发生率显著高于抗Jo-1阴性患者(分别为38.6%vs.20.0%,P=0.041;58.6%vs.38.2%,P=0.037),抗Jo-1抗体阳性患者肌酸激酶水平升高更为显著[982.5(175.8,1883.8)U/L vs.194.0(63.0,634.5)U/L,P=0.002],而抗Jo-1阴性患者更易出现技工手及干咳(分别为70.0%vs.89.1%,P=0.018;47.1%vs.78.2%,P=0.001)。两类患者在诊断及随访中出现恶性肿瘤,抗Jo-1阴性患者合并的恶性肿瘤以肺癌最为多见。在间质性肺病模式上,抗PL-7抗体阳性患者更易表现为非特异性间质性肺病。该队列平均随访时间(65.4±51.1)个月,13例患者随访过程中死亡,主要死亡原因为间质性肺病(53.8%)及肺部感染(30.8%)。结论:不同的抗合成酶抗体阳性患者临床表现不尽相同,对不明原因间质性肺病患者,尤其合并有关节炎、技工手等,临床应重视肌炎自身抗体的筛查,有助于协助诊断及判断患者的预后。恶性肿瘤可以是本病的伴发疾病之一,病程中应重视对恶性肿瘤的筛查。Objective:To analyze and summarize the clinical characteristics and prognosis of idiopathic inflammatory myopathy patients with non-anti-Jo-1 antibody positive.Method:The general data,laboratory examination,disease manifestations and pulmonary imaging data of idiopathic inflammatory myopathy patients with positive antisynthetase antibodies who were hospitalized in the Department of Rheumatology,Zhongshan Hospital of Fudan University from January 2011 to June 2021 were retrospectively collected.During the observation period to death or June 1,2022,the patients were divided into anti-Jo-1 positive group and anti-Jo-1 negative group according to the type of antisynthetase antibody.The clinical features and prognosis of the two groups were compared.Result:A total of 125 patients with positive anti-synthetase antibodies were enrolled.There were 70,17,6,3 and 29 patients with anti-Jo-1 antibody,anti-PL-7 antibody,anti-PL-12 antibody,anti-OJ antibody and anti-EJ antibody respectively.The incidence of arthritis and myasthenia in anti-Jo-1 antibody positive patients was significantly higher than those in anti-Jo-1 antibody negative patients(38.6%vs.20.0%,P=0.041;58.6%vs.38.2%,P=0.037,respectively).The level of creatine kinase in anti-Jo-1 antibody positive patients was significantly higher than that in anti-Jo-1 antibody positive patients[982.5(175.8,1883.8)U/L vs.194.0(63.0,634.5)U/L,P=0.002].Patients with anti-Jo-1 antibody negative were more likely to have mechanic’s hand and dry cough(70.0%vs.89.1%,P=0.018;47.1%vs.78.2%,P=0.001).Malignancy occurred in the two groups during diagnosis and follow-up,and lung cancer was the most common malignancy in patients with anti-Jo-1 antibody negative group.In the pattern of interstitial lung disease,anti-PL-7 antibody positive were more likely to show non-specific interstitial lung disease.The average follow-up time of the cohort was(65.4±51.1)months,13 patients died during follow-up,the main causes of death were interstitial lung disease(53.8%)and pulmonary infection(30.8%).Con

关 键 词：特发性炎性肌病 抗合成酶综合征 预后 

分 类 号：R593.2[医药卫生—内科学]