抗黑色素瘤分化相关基因5抗体阳性皮肌炎的临床和胸部CT表现

Clinical and chest CT manifestations of anti-melanoma differentiation-associated gene 5 antibody positive dermatomyositis

目的:探讨抗黑色素瘤分化相关基因5(MDA5)抗体阳性皮肌炎患者的临床表现及胸部损害的CT表现。方法:回顾性分析2017年2月至2019年10月在河南科技大学第一附属医院确诊的15例抗MDA5抗体阳性皮肌炎患者的临床表现、实验室检查及胸部CT表现。实验室检查指标主要包括抗MDA5抗体、抗Ro-52抗体、肌酸激酶等。胸部CT表现主要分析肺内表现(小叶间隔增厚、小叶内间质增厚、支气管血管束异常、磨玻璃影、胸膜下弧线、实变影、肺实质带、胸膜下间质增厚、牵引性支气管扩张)及肺外表现(腋窝淋巴结肿大、纵隔气肿、胸腔积液)。结果:12例有皮疹表现,8例有口腔、皮肤溃疡表现。实验室检查显示15例抗MDA5抗体均为阳性,12例抗Ro-52抗体阳性,2例肌酸激酶升高。CT示15例患者均有肺间质性改变,其中10例以间质性病变为主,4例肺间质性改变和肺实质病变并存,1例以肺实质性病变为主;11例有磨玻璃影,10例有小叶间隔增厚,7例有支气管血管束异常,9例有胸膜下弧线,5例小叶内间质增厚。3例有腋窝淋巴结肿大,1例有纵隔气肿,3例有胸腔积液;无肺间质纤维化病例。结论:多数抗MDA5抗体阳性皮肌炎患者临床上有皮疹或口腔、体表皮肤溃疡表现,其胸部CT表现以肺间质性病变为主,磨玻璃影和小叶间隔增厚发生率高,支气管血管束异常、胸膜下弧线及小叶内间质增厚3种征象可同时存在。

Objective To explore the clinical and chest CT features of anti-melanoma differentiation-associated gene 5(MDA5)antibody positive dermatomyositis.Methods Fifteen patients confirmed with anti-MDA5 antibody positive dermatomyositis in the First Affiliated Hospital of Henan University of Science and Technology from February 2017 to October 2019 were included in this study.The clinical data,laboratory results and CT images of the patients were retrospectively reviewed.The laboratory indicators included anti-MDA5 antibody,anti-Ro-52 antibody and creatine kinase.As for the CT imaging features,the pulmonary manifestations(interlobular septal thickening,intralobular interstitial thickening,peribronchovascular interstitial thickening,ground glass opacity,subpleural arc,consolidation,lung parenchymal zone,subpleural interstitial thickening,tractive bronchiectasis)and extrapulmonary manifestations(axillary lymph node enlargement,mediastinal emphysema,pleural effusion)were reviewed and recorded.Results Twelve cases had rash and 8 cases had oral and skin ulcers.Laboratory tests showed 15 cases with anti-MDA5 antibody positive,12 cases with anti-Ro-52 antibody positive,and 2 cases with creatine kinase increased.CT showed that 15 patients had pulmonary interstitial changes,of which 10 cases were mainly interstitial lesions,4 cases had pulmonary interstitial changes and pulmonary parenchyma lesions,1 case was mainly pulmonary parenchyma lesions.Eleven cases had ground glass opacity,10 cases had lobular septal thickening,7 cases had bronchial vascular bundle abnormalities,9 cases had subpleural arc,5 cases had lobular interstitial thickening.Three cases had axillary lymph node enlargement,1 case had mediastinal emphysema,3 cases had pleural effusion;No case of pulmonary fibrosis.Conclusions Most patients with anti-MDA5 antibody positive dermatomysitis have rash or oral cavity and skin ulcer.CT features are mainly presented as interstitial lung disease.Ground glass opacity and lobular septal thickening are common.Bronchial vascular bundle abnormalities,subpleural arc and lobular interstitial thickening can coexist in this disease.