Division of Pulmonary and Critical Care Medicine, Department of Medicine, UT Southwestern Medical Center, Dallas, TX, USA.
Department of Radiology and Division of Pulmonary and Critical Care Medicine, Department of Medicine, UT Southwestern Medical Center, Dallas, TX, USA.
Respir Med. 2021 Jul;183:106400. doi: 10.1016/j.rmed.2021.106400. Epub 2021 Apr 26.
Acute exacerbations of fibrosing interstitial lung disease (ILD) occur in both idiopathic pulmonary fibrosis (IPF) as well as non-IPF ILDs. An expert consensus definition has allowed for more frequent reporting of IPF exacerbations. The same is lacking for non-IPF ILD exacerbations. The incidence of non-IPF ILD exacerbations is likely less than in IPF, but the two entities share similar risk factors, such as increased frequency as physiologic derangements advance. The radiologic and histopathologic spectrum of acute ILD exacerbations extends from organizing pneumonia (OP) to the more treatment-refractory diffuse alveolar damage (DAD) pattern. Indeed, responsiveness to various therapies may depend on the relative components of these entities, favoring OP over DAD. There are no proven therapies for acute ILD exacerbations. Corticosteroids are a mainstay in any regimen although clear evidence of benefit does not exist. A variety of immunosuppressant agents have purported success in historical cohort studies - cyclophosphamide, cyclosporine A, and tacrolimus most commonly. Only one randomized controlled trial has been published, studying recombinant thrombomodulin for IPF exacerbation, but the primary outcome of survivor proportion at 90 days was not met. Other novel therapies for ILD exacerbations are still under investigation. The short and long-term prognosis of acute exacerbations of ILD is poor, especially in patients with IPF. Transplant referral should be considered early for both IPF as well as fibrosing non-IPF ILDs, given the unpredictability of the exacerbation event.
纤维化性间质性肺疾病（ILD）的急性加重在特发性肺纤维化（IPF）和非特发性ILD 中均会发生。专家共识定义使得 IPF 加重的报告更为频繁。而非特发性ILD 加重则缺乏类似的定义。非特发性ILD 加重的发生率可能低于 IPF，但这两种疾病具有相似的危险因素，例如随着生理紊乱的进展，其发生频率会增加。急性ILD 加重的放射学和组织病理学谱从机化性肺炎（OP）扩展到更难治疗的弥漫性肺泡损伤（DAD）模式。实际上，对各种治疗的反应可能取决于这些疾病的相对成分，OP 比 DAD 更有反应性。目前尚无针对急性ILD 加重的有效治疗方法。皮质类固醇是任何方案的主要药物，尽管其疗效尚无明确证据。各种免疫抑制剂在历史队列研究中都被认为有一定疗效，最常用的是环磷酰胺、环孢素 A 和他克莫司。仅有一项关于 IPF 加重的重组血栓调节蛋白的随机对照试验已发表，但 90 天的生存比例主要终点未达到。ILD 加重的其他新型治疗方法仍在研究中。ILD 急性加重的短期和长期预后均较差，尤其是在 IPF 患者中。鉴于加重事件的不可预测性，对于 IPF 和纤维化性非特发性ILD，均应早期考虑进行移植转诊。
