本文由小咖机器人翻译整理

期刊来源：JAMA

原文链接：https://doi.org/10.1001/jama.2024.3669

摘要内容如下：

重要性

间质性肺疾病（ILD）是一组以肺实质炎症和/或纤维化为特征的肺部疾病，伴有进行性呼吸困难，常导致终末期呼吸衰竭。在美国，ILD每年影响约650，000人，并导致约25，000至30，000人死亡。

观察

ILD最常见的形式是特发性肺纤维化（IPF），约占所有ILD病例的三分之一；过敏性肺炎，占ILD病例的15%；结缔组织病（CTD），占ILD病例的25%。ILD通常表现为用力时呼吸困难。大约30%的ILD患者报告咳嗽。胸部计算机断层扫描诊断ILDS亚型（如IPF）的敏感性约为91%，特异性约为71%。生理评估提供了重要的预后信息。12个月内用力肺活量（FVC）下降5%，与FVC无变化相比，死亡率增加约2倍。尼替达尼或吡非尼酮抗纤维化治疗可使IPF、硬皮病相关ILD患者和任何原因的进行性肺纤维化患者的年FVC下降减缓约44%至57%。对于结缔组织病相关的ILD，免疫调节治疗，如托珠单抗、利妥昔单抗和霉酚酸酯，在12个月的随访中可能会减缓甚至改善FVC的下降。结构性运动疗法可减轻症状并改善呼吸困难患者的6分钟步行测试距离。在6分钟步行测试中饱和度低于88%的ILD患者中，氧气可减轻症状并改善生活质量。肺移植可以改善终末期ILD患者的症状并解决呼吸衰竭。肺移植后，ILD患者的中位生存期为5.2至6.7年，而未接受肺移植的晚期ILD患者的中位生存期不到2年。高达85%的终末期纤维化ILD患者会发展为肺动脉高压。在这些患者中，吸入曲前列环素治疗可改善行走距离和呼吸道症状。

结论和相关性

间质性肺疾病通常表现为用力时呼吸困难，并可发展为呼吸衰竭。一线治疗包括用于IPF的尼替达尼或吡非尼酮，以及用于结缔组织病所致ILD的霉酚酸酯。晚期ILD患者应考虑肺移植。在ILD患者中，运动训练可改善6分钟步行测试距离和生活质量。

英文原文如下：

Abstracts

Importance  Interstitial lung disease (ILD) consists of a group of pulmonary disorders characterized by inflammation and/or fibrosis of the lung parenchyma associated with progressive dyspnea that frequently results in end-stage respiratory failure. In the US, ILD affects approximately 650 000 people and causes approximately 25 000 to 30 000 deaths per year.

Observations  The most common forms of ILD are idiopathic pulmonary fibrosis (IPF), which accounts for approximately one-third of all cases of ILD, hypersensitivity pneumonitis, accounting for 15% of ILD cases, and connective tissue disease (CTD), accounting for 25% of ILD cases. ILD typically presents with dyspnea on exertion. Approximately 30% of patients with ILD report cough. Thoracic computed tomography is approximately 91% sensitive and 71% specific for diagnosing subtypes of ILDs such as IPF. Physiologic assessment provides important prognostic information. A 5% decline in forced vital capacity (FVC) over 12 months is associated with an approximately 2-fold increase in mortality compared with no change in FVC. Antifibrotic therapy with nintedanib or pirfenidone slows annual FVC decline by approximately 44% to 57% in individuals with IPF, scleroderma associated ILD, and in those with progressive pulmonary fibrosis of any cause. For connective tissue disease-associated ILD, immunomodulatory therapy, such as tocilizumab, rituximab, and mycophenolate mofetil, may slow decline or even improve FVC at 12-month follow-up. Structured exercise therapy reduces symptoms and improves 6-minute walk test distance in individuals with dyspnea. Oxygen reduces symptoms and improves quality of life in individuals with ILD who desaturate below 88% on a 6-minute walk test. Lung transplant may improve symptoms and resolve respiratory failure in patients with end-stage ILD. After lung transplant, patients with ILD have a median survival of 5.2 to 6.7 years compared with a median survival of less than 2 years in patients with advanced ILD who do not undergo lung transplant. Up to 85% of individuals with end-stage fibrotic ILD develop pulmonary hypertension. In these patients, treatment with inhaled treprostinil improves walking distance and respiratory symptoms.

Conclusions and Relevance  Interstitial lung disease typically presents with dyspnea on exertion and can progress to respiratory failure. First-line therapy includes nintedanib or pirfenidone for IPF and mycophenolate mofetil for ILD due to connective tissue disease. Lung transplant should be considered for patients with advanced ILD. In patients with ILD, exercise training improves 6-minute walk test distance and quality of life.

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