多发性肌炎和皮肌炎相关间质性肺疾病的临床特征分析

Clinical characteristics analysis of polymyositis/dermatomyositis related interstitial lung disease

目的了解多发性肌炎(polymyositis,PM)和皮肌炎(dermatomyositis,DM)相关间质性肺疾病(interstitial lung disease,ILD)及其不同亚型的临床特征。方法回顾性分析2015年1月至2020年12月北京积水潭医院收治的14例PM/DM-ILD患者的临床特征,分析基于抗体分型的不同PM/DM亚型的ILD特点及急进型ILD病情进展的因素。结果PM-ILD患者表现出典型的近端肌无力症状,而DM-ILD患者表现多样,但均有特征性皮疹,DM-ILD较PM-ILD更易表现为急性间质性肺炎。不同PM/DM抗体亚型的ILD临床表现有差异,抗合成酶抗体阳性的PM/DM亚型相关性ILD发生率虽高,但预后一般较好;而抗MDA5亚型的DM相关性ILD多表现为急进型ILD,进展快,预后差。急进型ILD病情恶化主要原因是急性间质性肺炎,而非感染、心力衰竭及药物诱发性肺炎。结论PM/DM-ILD不同亚型临床特征差别大,抗MDA5亚型多进展为急性间质性肺炎,且预后差,因此明确亚型并分析急进型ILD的进展原因很重要。

Objective To understand the clinical characteristics of different subtypes of polymyositis(PM)/dermatomyositis(DM) related interstitial lung disease(ILD). Methods Fourteen cases of PM/DM related ILD admitted in Beijing Jishuitan Hospital from Jan 2015 to Dec 2020 were retrospectively included. The characteristics of ILD of different subtypes of PM/DM classified based on antibody examination, as well as the risk factors of the progression of aggressive type ILD, were analyzed. Results Patients with PM-ILD presented classical proximal skeletal muscle weakness. While patients with DM-ILD had more nonspecific symptoms but all patients had the typical skin erythema. Acute interstitial pneumonia in DM-ILD was more common than in PM-ILD. The clinical manifestations varied with the antibody subtypes of PM/DM-ILD. Patients with the antisynthetase antibody-positive subtype had a high incidence of ILD but a good prognosis.Patients with MDA-5 antibody-positive subtype DM had a high incidence of the aggressive type ILD with a rapid progression and a poor prognosis. The main reason for the deterioration of the aggressive type ILD was acute interstitial pneumonia rather than infection, heart failure or drug-induced pneumonia. Conclusions The clinical manifestations of different subtypes of PM/DM-ILD vary tremendously. Patients with MDA-5 antibody-positive subtype has a high incidence of progression to acute interstitial pneumonia with a poor prognosis. Therefore, it is important to recognize the subtype diagnosis and the reasons for the progression of the aggressive type ILD.