Lange Steven M., Sankari Abdulghani, Parekh Maansi
Thomas Jefferson University Hospital
Wayne State University
Interstitial lung disease (ILD) refers to different manifestations of pulmonary disorders due to inflammation and/or fibrosis of the pulmonary parenchyma. Collagen vascular disease is a common type of ILD etiology and encompasses a diverse group of immunologically mediated entities that share overlapping clinical and histopathologic features as well as manifest in characteristic patterns of ILD. The collagen vascular diseases which commonly affect the pulmonary system include rheumatoid arthritis (RA), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), polymyositis (PM), dermatomyositis (DM), mixed connective tissue disease (MCTD), and Sjogren’s syndrome (SS). The degree of lung involvement and spectrum of thoracic findings vary among the disorders, may be seen with clinically early or late disease, and portends certain patient outcomes. Identification of pulmonary involvement has important therapeutic and prognostic implications. Collagen vascular disease may be associated with usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), diffuse alveolar damage (DAD), and lymphocytic interstitial pneumonia (LIP). This article will review the etiology, epidemiology, pathogenesis, history and examination, evaluation, management, and prognosis of ILD related to collagen vascular disease.
间质性肺疾病（ILD）是指由于肺实质的炎症和/或纤维化导致的肺部疾病的不同表现。胶原血管病是ILD病因的一种常见类型，包括一组免疫介导的疾病，它们具有重叠的临床和组织病理学特征，并以特征性的ILD模式表现出来。通常影响肺部系统的胶原血管病包括类风湿性关节炎（RA）、系统性硬化症（SSc）、系统性红斑狼疮（SLE）、多发性肌炎（PM）、皮肌炎（DM）、混合性结缔组织病（MCTD）和干燥综合征（SS）。不同疾病的肺部受累程度和胸部表现范围各不相同，可在疾病的临床早期或晚期出现，并预示着特定的患者预后。识别肺部受累具有重要的治疗和预后意义。胶原血管病可能与寻常型间质性肺炎（UIP）、非特异性间质性肺炎（NSIP）、隐源性机化性肺炎（COP）、弥漫性肺泡损伤（DAD）和淋巴细胞间质性肺炎（LIP）相关。本文将综述与胶原血管病相关的ILD的病因、流行病学、发病机制、病史与检查、评估、管理及预后。
