中国人群中多发性肌炎的发病率及临床特征  

Prevalence and clinical characteristics of polymyositis in a Chinese cohort

作　　者：孙超 田小兰[2] 杨红霞 杨阚波 李珊珊[2] 江薇[2] 彭清林[2] 王国春[2] 卢昕[2] Sun Chao;Tian Xiaolan;Yang Hongxia;Yang Hanbo;Li Shanshan;Jiang Wei;Peng Qinglin;Wang Guochun;Lu Xin(Peking University,China-Japan Friendship School of Clinical Medicine,Beijing 100029,China;Department of Rheumatology,China-Japan Friendship Hospital,Beijing 100029,China)

机构地区：[1]北京大学中日友好临床医学院,北京100029 [2]中日友好医院风湿免疫科,北京100029

出　　处：《中华风湿病学杂志》2023年第3期158-163,I0002,共7页Chinese Journal of Rheumatology

基　　金：北京市科学技术委员会首都临床诊疗技术研究与示范应用项目(Z191100006619012)。

摘　　要：目的分析特发性炎性肌病(IIM)中多发性肌炎(PM)的发生率及其特征,探讨IIM中PM是否被过度诊断。方法纳入2008—2019年于中日友好医院风湿免疫科住院依据Bohan与Peter标准诊断的所有IIM患者。确定诊断的PM(definite PM,dPM)定义为具有典型的PM临床特征和病理特征,包括肌酸激酶(CK)升高和肌无力,肌活检呈现肌细胞膜表达MHC-I阳性和CD8+T细胞浸润肌内膜。同时,参照IIM各亚型的最新诊断标准,排除DM、抗合成酶综合征(ASS)、免疫介导的坏死性肌病(IMNM)、散发性包涵体肌炎和其他肌病。应用SPSS 24.0进行统计学分析。Kruskal-Wallis检验和χ^(2)检验用于比较dPM组和其他IIM亚型患者临床特点的差异。结果共1259例IIM纳入研究,其中1015例(80.6%)为DM,244例(19.4%)为PM。参照严格定义的dPM标准,IIM中仅有0.5%(6/1259)的患者可被诊断为dPM。原先诊断为PM的患者在新的IIM亚型分类中多数为IMNM和ASS,其中48.0%(117/244)为IMNM,32.0%(78/244)为ASS。6例dPM患者中女性4例,占66.7%,其中1例合并RA,1例合并SSc。所有dPM患者均有肌无力,轻度CK升高[611(391,1451)]U/L,肌炎特异性自身抗体均为阴性。除了1例dPM患者因合并慢性阻塞性肺疾病,未接受免疫调节治疗外,其余5例患者均予低/中等剂量糖皮质激素联合或不联合免疫抑制剂治疗。在随访(38±26)个月后,患者肌力均改善。结论PM是一类非常罕见的IIM临床亚型,PM在临床上存在过度诊断。严格定义的PM是一类临床症状轻,预后好的疾病。Objective To analyze the frequency and characteristics of polymyositis(PM)in idiopathic inflammatory myopathy(IIM),and to investigate whether PM is over-diagnosed.Methods Patients diagnosed as IIM according to the Bohan&Peter criteria of IIM hospitalized in the Department of Rheumatology of China-Japan Friendship Hospital from 2008 to 2019 were involved in the study.Definite PM(dPM)was defined as typical clinical and pathological features including elevated creatine kinase(CK)level,muscle weakness and muscle biopsy findings with endomysial CD8+T cell infiltration and expression of MHC-1 on sarcolemma.Meanwhile,dermatomyositis(DM),anti-synthase syndrome(ASS),immune-mediated necrotic myopathy(IMNM),sporadic inclusion body myositis(sIBM)and other myopathies were excluded according to the new classification criteria of IIM subtypes respectively.Statistical analysis was performed using SPSS software 24.0.The Kruskal-Wallis test andχ^(2) test were used to compare the clinical characteristics between the dPM group and other IIM subtypes.Results A total of 1259 patients with IIM including 1015(80.6%)DM and 244(19.4%)PM were enrolled in this study.According to the strict definition of PM criteria,only 0.5%of patients(6/1259)in IIM could be diagnosed as dPM.Most PM patients were IMNM and ASS according to the new IIM subtypes criteria,of which 48.0%(117/244)were IMNM and 32.0%(78/244)were ASS.66.7%(4/6)of dPM patients were women.One complicated with RA,and one was dPM overlaped with systemic sclerosis.All of them had muscle weakness,mild elevation of CK level[611(391,1451)U/L],and were myositis-specific autoantibodies negative.Except one dPM patients who did not receive immunoregulatory therapy due to chronic obstructive pulmonary disease,the others were administrated with low or medium dose prednisone combined with or without immunosuppressive agents.After a median follow-up of(38±26)months,the muscle strength of dPM patients were improved.Conclusion dPM is a very rare clinical subtype of IIM.PM is an over-diagnosed enti

关 键 词：多发性肌炎 特发性炎性肌病 过度诊断 

分 类 号：R593.26[医药卫生—内科学]