免疫介导的坏死性肌病重叠综合征患者的临床特点分析

Clinical features of immune-mediated necrotizing myopathy overlap syndrome

目的 总结归纳免疫介导的坏死性肌病(IMNM)重叠综合征患者的临床特点。方法 收集2011年至2020年武汉同济医院确诊的15例重叠综合征患者,根据重叠不同结缔组织病(CTD)分为IMNM重叠干燥综合征(SS)组(10例)、重叠类风湿性关节炎(RA)组(4例)、重叠系统性红斑狼疮(SLE)组(1例)。对3组患者的临床表现、实验室检查、治疗和预后等资料进行分析。结果IMNM-SS组患者中女性发病占比较高;血清肌酸激酶(CK)平均水平(3129±4719)u/L,在3组中最高。IMNM-RA组患者平均病程(3.7±1.7)个月,在3组中最短。IMNM-SLE组患者平均发病年龄23岁,在3组中最年轻。IMNM-SS和IMNM-RA组患者间质性肺病(ILD)和心脏功能受累比例高;血清肌炎抗体以抗SRP、抗SS-A/Ro-52抗体表达比例最高。3组患者多采用激素联合免疫抑制剂治疗方法(IMNM-SS患者占比80%;IMNM-RA患者占比75%;IMNM-SLE患者占比100%),其中47.6%患者采用了糖皮质激素联合他克莫司治疗。2例(13.3%)患者基本恢复正常,12例(80%)患者病情得到好转。结论 不同类型重叠综合征患者临床特异性症状不显著。IMNM-SS和IMNM-RA患者合并心脏和(或)肺部损害比例较高,建议临床尽早完善患者心脏和肺部检查。多数患者采用激素联合免疫抑制剂治疗后临床症状好转,预后较好。

Objective To investigate the clinical features of patients with immune-mediated necrotizing myopathy(IMNM) overlap syndrome. Methods A total of 15 patients with overlap syndrome who were diagnosed in Tongji Hospital from 2011 to 2020 were enrolled, and according to the type of connective tissue disease overlapped, they were divided into IMNM-Sjogren’s syndrome(SS) group with 10 patients, IMNM-rheumatoid arthritis(RA) group with 4 patients, and IMNMsystemic lupus erythematosus(SLE) group with 1 patient. The three groups were analyzed in terms of clinical manifestation, laboratory examination, treatment, and prognosis. Results The IMNM-SS group had a relatively high proportion of female patients and the highest serum creatine kinase level of 3129±4719 u/L among the three groups. The IMNM-RA group had a mean course of disease of 3.7±1.7 months, which was the shortest among the three groups. The IMNM-SLE group had a mean age of onset of 23 years, which was the youngest among the three groups. The IMNM-SS and IMNM-RA groups had a relatively high proportion of patients with interstitial lung disease or cardiac involvement and the highest proportion of the patients with the expression of anti-SRP and anti-SS-A/Ro-52 antibodies among serum myositis antibodies. Most patients in the three groups received glucocorticoid combined with immunosuppressant, accounting for 80% in the IMNM-SS group, 75%in the IMNM-RA group, and 100% in the IMNM-SLE group, and 47.6% of these patients were treated with glucocorticoids combined with tacrolimus. Of all patients, 2 patients(13.3%) basically returned to normal and 12(80%) had achieved marked improvement. Conclusions There is a lack of marked specific clinical symptoms for different types of overlap syndrome. IMNM-SS and IMNM-RA tend to have a high proportion of patients with cardiac and/or pulmonary injury, and it is recommended to perform heart and lung examinations as early as possible in clinical practice. Most patients can achieve the improvement in clinical symtomps and have good prognosis after the treatment with glucocorticoid and immunosuppressant.