摘要
目的探索抗黑色素瘤分化相关基因5(melanoma differentiation-associated gene 5,MDA5)抗体阳性的特发性炎症性肌病(idiopathic inflammatory myopathy,IIM)合并间质性肺疾病(interstitial lung disease,ILD)的临床特征。方法收集2019年1月—2022年3月于山西白求恩医院住院的91例IIM患者。根据抗MDA5抗体结果(阳性/阴性)及是否合并ILD分组,回顾性分析不同组间患者的临床症状、实验室检查和影像学特点等。结果抗MDA5抗体阳性组Gottron征(71.0%比38.3%)、溃疡性皮疹(45.2%比16.7%)、呼吸困难(64.5%比36.7%)、ILD(71.0%比43.3%)的发生率与阴性组相比差异有统计学意义(均P<0.05)。抗MDA5抗体阳性组肺高分辨CT磨玻璃影(81.8%)与阴性组(53.8%)相比差异有统计学意义(P<0.05)。抗MDA5抗体阳性IIM合并ILD组Gottron征发生率(77.3%比33.3%)、乳酸脱氢酶[(525.20±203.09)IU/L比(321.73±188.88)IU/L]、D-二聚体[(1226.65±902.31)U/L比(703.73±160.11)U/L]、人涎液化糖链抗原6[(1399.55±869.64)U/mL比(371.56±128.92)U/mL]水平、抗Ro-52抗体阳性率(68.2%比22.3%)及病死率(54.5%比0.0%)与非ILD组相比差异有统计学意义(均P<0.05)。经大剂量糖皮质激素联合环磷酰胺及另外一种免疫抑制剂(他克莫司或托法替布)的三联治疗后,病死率较既往大剂量糖皮质激素联合环磷酰胺治疗有所下降(28.6%比47.1%)。结论抗MDA5抗体阳性IIM合并ILD患者最常见的临床表现是皮损和呼吸困难,磨玻璃影是最常见的影像学表现,合并快速进展性ILD后病死率高,早期强化免疫抑制治疗可能有助于提高患者生存率。
Objective This study aims to analyze the clinical characteristics of idiopathic inflammatory myopathy(IIM)associated interstitial lung disease(ILD)with positive anti melanoma differentiation-associated gene 5(MDA5)antibody.Methods The clinical data of 91 patients with IIM in Shanxi Bethune Hospital from Jan 2019 to Mar 2022 were collected.Then various comparisons and analyzed retrospectively in the clinical symptoms,laboratory indicators and imaging characteristics between the negative MDA5-IIM and the positive anti-MDA5-IIM.Results Compared with the negative anti-MDA5-IIM patients,the significantly higher incidences of Gottron sign(71.0%vs.38.3%),ulcerative rash(45.2%vs.16.7%),dyspnea(64.5%vs.36.7%),and ILD(71.0%vs.43.3%),in the positive anti-MDA5-IIM patients(P<0.05).Compared with the negative anti-MDA5-IIM patients,the more ground-glass opacification(GGO)in the positive anti-MDA5-IIM patients on high resolution computed tomography(81.8%vs.53.8%,P<0.05).Compared with the positive anti-MDA5-IIM patients without ILD,the positive anti-MDA5-IIM patients with ILD had significantly higher incidence of Gottron sign(77.3%vs.33.3%),the increased levels of lactate dehydrogenase[(525.20±203.09)IU/L vs.(321.73±188.88)IU/L],Krebs von den Lungen-6[(1399.55±869.64)U/mL vs.(371.56±128.92)U/mL],and D-dimer[(1226.65±902.31)U/L vs.(703.73±160.11)U/L],as well as higher positive rate of anti-Ro-52 antibody(68.2%vs.22.2%)and the higher case fatality rate(54.5%vs.0.0%),with all P<0.05.The triple treatment of highdose glucocorticoid(GC)combined with intravenous cyclophosphamide(IVCY)and another immunosuppressive agent(tacrolimus or tofacitinib)was found to be effective in the MDA5-IIM associated ILD group,with a lower case fatality rate(28.6%)than high-dose GC combined with IVCY group(47.1%).Conclusions The positive anti-MDA5-IIM with ILD of patients have the higher incidence of rash and dyspnea,with GGO as the most common imaging manifestation.When rapidly progressive interstitial lung disease(RPILD)is combined,the case fatalit
作者
王鹏飞
郝斌威
江小鹏
段炜
WANG Pengfei;HAO Binwei;JIANG Xiaopeng;DUAN Wei(Department of Respiratory and Critical Care Medicine,Shanxi Bethune Hospital(Shanxi Academy of Medical Sciences),Taiyuan,Shanxi 030032,P.R.China;Department of Radiology,Shanxi Bethune Hospital(Shanxi Academy of Medical Sciences),Taiyuan,Shanxi 030032,P.R.China)
出处
《中国呼吸与危重监护杂志》
CAS
CSCD
北大核心
2023年第4期245-250,共6页
Chinese Journal of Respiratory and Critical Care Medicine
关键词
间质性肺疾病
特发性炎症性肌病
抗黑色素瘤分化相关基因5抗体
临床特征
治疗
Interstitial lung disease
idiopathic inflammatory myositis
anti-melanoma differentiation associated gene 5
clinical characteristics
therapy
