Solomon Joshua J, Fischer Aryeh
Department of Medicine, National Jewish Health, Denver, Colorado, USA.
Department of Medicine, National Jewish Health, Denver, Colorado, USA
J Intensive Care Med. 2015 Oct;30(7):392-400. doi: 10.1177/0885066613516579. Epub 2013 Dec 25.
The connective tissue diseases (CTDs) are a group of systemic disorders characterized by autoimmunity and autoimmune-mediated organ damage. The lung is a frequent target and all components of the respiratory system are at risk. Interstitial lung disease (ILD) represents a broad group of diffuse parenchymal lung injury patterns characterized by varying degrees of inflammation and fibrosis, is a common manifestation of CTD particularly common in systemic sclerosis, polymyositis/dermatomyositis, and rheumatoid arthritis, and is a leading cause of significant morbidity and mortality. The lung injury patterns of CTD-associated ILD (CTD-ILD) mirror those of idiopathic interstitial pneumonia and may arise at any time during the course of the CTD or may be the first manifestation of CTD. Patients with CTD that present with respiratory failure often present significant diagnostic dilemmas. Thorough and comprehensive assessments to exclude respiratory *infection, acute interstitial pneumonia, medication toxicity, pulmonary embolism, cardiac dysfunction, and diffuse alveolar hemorrhage are the fundamental components for the evaluation of such patients. Furthermore, patients with CTD are also at risk of acute exacerbations of underlying ILD. Acute exacerbations are manifested by subacute respiratory deterioration with worsening hypoxemia in the setting of new radiographic abnormalities. The prognosis of patients with CTD having respiratory failure is often quite poor, highlighting the need for prompt and thorough clinical assessments to determine the underlying etiology and implementation of appropriate therapeutic strategies.
结缔组织病（CTD）是一组以自身免疫和自身免疫介导的器官损害为特征的全身性疾病。肺是常见的受累器官，呼吸系统的所有组成部分都有风险。间质性肺疾病（ILD）是一大类以不同程度的炎症和纤维化为特征的弥漫性实质性肺损伤模式，是CTD的常见表现，在系统性硬化症、多发性肌炎/皮肌炎和类风湿关节炎中尤为常见，是导致严重发病和死亡的主要原因。CTD相关ILD（CTD-ILD）的肺损伤模式与特发性间质性肺炎相似，可能在CTD病程中的任何时候出现，也可能是CTD的首发表现。出现呼吸衰竭的CTD患者常常面临重大的诊断难题。全面彻底地评估以排除呼吸道感染、急性间质性肺炎、药物毒性、肺栓塞、心脏功能障碍和弥漫性肺泡出血是评估此类患者的基本要素。此外，CTD患者还面临潜在ILD急性加重的风险。急性加重表现为亚急性呼吸功能恶化，在出现新的影像学异常的情况下低氧血症加重。患有呼吸衰竭的CTD患者的预后通常很差，这突出表明需要及时、全面地进行临床评估以确定潜在病因并实施适当的治疗策略。
