Luo Xiaohui, Xiang Fei
Department of Pulmonary and Critical Care Medicine, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
J Thorac Dis. 2024 Jul 30;16(7):4727-4741. doi: 10.21037/jtd-23-1565. Epub 2024 Jul 11.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial pneumonia, which is the commonest type of idiopathic interstitial pneumonia in the clinic. For most patients, the course of the disease is slow and prolonged, but a percentage of them develop an acute respiratory worsening during the disease, known as an acute exacerbation of IPF (AE-IPF). The updated guidelines define AE-IPF as an acute worsening of dyspnea in an IPF patient within 1 month and exclude other conditions such as left heart failure and pulmonary embolism. However, the prevention and treatment of AE-IPF are still unclear. Based on the high mortality rate caused by AE, in this article, we will focus on the latest research advances in AE-IPF treatment strategies and provide a comprehensive review of its pathogenesis, risk factors, clinical features, and diagnosis.
This study searched for relevant literature published from 2018 to 2023 in the PubMed database. The search terms used were as follows: "Acute exacerbation", "Idiopathic pulmonary fibrosis", "Biomarker", "Pathogenesis", "Treatment", "HRCT", "Antifibrotic", "Infection", "Immunosuppressant", "Autoantibody", "Oxygen therapy", "Hemoperfusion", "Inflammation".
The review found that corticosteroids are still the primary treatment strategy at present, although there is some controversy regarding the dosing and tapering of corticosteroids. However, corticosteroids combined with intravenous cyclophosphamide have been shown to be detrimental to the prognosis of patients with AE-IPF. Given its deadly high mortality rate, early intervention is crucial. Pirfenidone and nintedanib have been proven to reduce incidence of AE. Meanwhile, in the future, the lung microbiome may also be a break-through.
This study reviewed the pathogenesis and risk factors of AE-IPF and updated the current and potential treatment strategies regarding AE-IPF. The pathogenesis of AE-IPF is not exact, multiple mechanisms may be involved simultaneously. Corticosteroids remain the mainstream treatment modality in the medical treatment of AE-IFP. Many other treatment modalities have been proposed in succession, but no clear conclusions can be drawn about the effectiveness and safety of these interventions.
特发性肺纤维化（IPF）是一种慢性、进行性、纤维化间质性肺炎，是临床上最常见的特发性间质性肺炎类型。对大多数患者而言，疾病进程缓慢且持久，但其中一部分患者在疾病过程中会出现急性呼吸功能恶化，即所谓的特发性肺纤维化急性加重（AE-IPF）。最新指南将AE-IPF定义为IPF患者在1个月内出现的急性呼吸困难加重，并排除其他情况，如左心衰竭和肺栓塞。然而，AE-IPF的预防和治疗仍不明确。鉴于AE导致的高死亡率，在本文中，我们将重点关注AE-IPF治疗策略的最新研究进展，并对其发病机制、危险因素、临床特征及诊断进行全面综述。
本研究检索了2018年至2023年在PubMed数据库中发表的相关文献。使用的检索词如下：“急性加重”“特发性肺纤维化”“生物标志物”“发病机制”“治疗”“高分辨率CT”“抗纤维化”“感染”“免疫抑制剂”“自身抗体”“氧疗”“血液灌流”“炎症”。
综述发现，目前糖皮质激素仍是主要治疗策略，尽管在糖皮质激素的剂量和减量方面存在一些争议。然而，已表明糖皮质激素联合静脉注射环磷酰胺对AE-IPF患者的预后有害。鉴于其极高的死亡率，早期干预至关重要。吡非尼酮和尼达尼布已被证明可降低AE的发生率。同时，未来肺部微生物群也可能成为一个突破点。
本研究综述了AE-IPF的发病机制和危险因素，并更新了关于AE-IPF的当前及潜在治疗策略。AE-IPF的发病机制尚不确切，可能同时涉及多种机制。糖皮质激素仍然是AE-IPF药物治疗的主流方式。相继提出了许多其他治疗方式，但关于这些干预措施的有效性和安全性尚无明确结论。
