Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.
Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Clin Respir J. 2021 Mar;15(3):336-344. doi: 10.1111/crj.13304. Epub 2020 Dec 10.
Acute exacerbation (AE) is recognized as a life-threatening condition with acute respiratory worsening in idiopathic pulmonary fibrosis (IPF). AE also occurs in fibrotic interstitial lung disease (ILD) other than IPF, including other types of idiopathic interstitial pneumonias (IIPs), ILD associated with collagen vascular disease (CVD-ILD), and chronic hypersensitivity pneumonia (CHP). However, the clinical impact after AE in those patients is still unclear.
A retrospective review of 174 consecutive first-episodes with AE of ILD in our institution from 2002 to 2016 was performed. AE was defined according to the revised definition and diagnostic criteria proposed by an international working group in 2016. Clinical characteristics, 90-day survival, and the requirement of long-term oxygen therapy (LTOT) after AE were evaluated in each underlying ILD.
There were 102 patients with AE of IPF (AE-IPF) and 72 with AE of ILD other than IPF, including non-IPF IIPs (n = 29) and secondary ILD (n = 43) [CVD-ILD (n = 39), CHP (n = 4)]. In CVD-ILD, rheumatoid arthritis (n = 17) was most common. The 90-day mortality after AE was 57% in IPF, 29% in non-IPF IIPs, and 33% in secondary ILD. After AE, ILD other than IPF had a significantly better survival rate than IPF (P < 0.001). Among survivors, the rates of patients requiring LTOT after AE were 63% in IPF, 35% in non-IPF IIPs, and 46% in secondary ILD, respectively.
AE of ILD other than IPF might have a better prognosis than AE-IPF, but both are fatal conditions that cause chronic respiratory failure.
急性加重（AE）被认为是一种危及生命的疾病，其特征是特发性肺纤维化（IPF）患者的急性呼吸恶化。AE 也发生在除 IPF 以外的纤维化间质性肺疾病（ILD）中，包括其他类型的特发性间质性肺炎（IIP）、与结缔组织疾病相关的ILD（CVD-ILD）和慢性过敏性肺炎（CHP）。然而，这些患者 AE 后的临床影响仍不清楚。
对 2002 年至 2016 年我院 174 例ILD 首次 AE 患者进行回顾性分析。AE 根据 2016 年国际工作组提出的修订定义和诊断标准进行定义。评估每种ILD 的临床特征、90 天生存率和 AE 后长期氧疗（LTOT）的需求。
102 例 AE-IPF（AE-IPF）和 72 例ILD 以外的 AE 患者，包括非 IPF IIP（n=29）和继发性ILD（n=43）[CVD-ILD（n=39）、CHP（n=4）]。在 CVD-ILD 中，最常见的是类风湿关节炎（n=17）。AE 后 90 天死亡率在 IPF 中为 57%，在非 IPF IIP 中为 29%，在继发性 ILD 中为 33%。AE 后，ILD 以外的疾病生存率明显优于 IPF（P<0.001）。在存活患者中，AE 后需要 LTOT 的患者比例分别为 IPF 中的 63%、非 IPF IIP 中的 35%和继发性 ILD 中的 46%。
ILD 以外的 AE 可能比 AE-IPF 预后更好，但两者都是导致慢性呼吸衰竭的致命疾病。
