结缔组织病相关间质性肺病的诊治策略

结缔组织病(connective tissue disease,CTD)是一组全身性自身免疫性疾病,病变累及多种脏器。由于肺和胸膜均富含胶原、血管等结缔组织,因此 CTD 大多可以损伤肺和胸膜等呼吸系统多个器官,包括：呼吸肌、胸膜、肺血管、气道、肺实质和肺间质,且部分患者呼吸道表现为首发症状。间质性肺病(interstitial lung disease,ILD)在 CTD 中十分常见,发生率在数个 CTD 病种中超过50%。与之相对应,15%~30%初诊为特发型间质性肺炎(idiopathic interstitial pneumonia,IIP)的患者最终被确认符合 CTD-ILD 诊断。ILD 是导致 CTD 患者死亡的重要原因之一。CTD 包括包括系统性红斑狼疮(SLE)、类风湿性关节炎(RA)、原发性干燥综合征(pSS)、多发性肌炎(PM)、皮肌炎(DM)、系统性硬化(SSc)和混合性 CTD (MCTD)等。本文就 CTD-ILD 的诊治策略作一简要综述。

Interstitial lung disease associated with connective tissue disease:strategy of diagnosis and treatment

Connective tissue disease(CTD) is a systemic autoimmune disease,which can damage multiple organs.Due to the lung and pleural are rich in collagen,blood vessels and other connective tissue, therefore,CTD can damage the lungs and other organs of the respiratory system which includes respiratory muscle,pleural,pulmonary vascular,airway,parenchyma and interstitial lung.Some of the CTD patients with respiratory symptoms are the first symptom.Interstitial lung disease (ILD) is very common in CTD.The total incidence rates are more than 50%.In contrast,1 5% to 30% patients with idiopathic interstitial pneumonia(IIP)are finally confirmed to be consistent with CTD-ILD diagnosis.ILD is one of the important causes of death in CTD patients.CTD include systemic lupus erythematosus (SLE),rheumatoid arthritis (RA),primary Sjogren syndrome (pSS),polymyositis (PM), dermatomyositis (DM),systemic sclerosis (SSc) and mixed CTD (MCTD).In this pater,the strategy of diagnosis and treatment for CTD-ILD were reviewed.