Acute exacerbations of fibrotic interstitial lung diseases
Acute exacerbations of fibrotic interstitial lung diseases
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DOI：
10.1111/resp.13682
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发表时间：
2019-08-19
期刊：
影响因子：
6.9
通讯作者：
Hasegawa, Yoshinori
中科院分区：
文献类型：
--
作者：
Suzuki, Atsushi;Kondoh, Yasuhiro;Hasegawa, Yoshinori
背景和目的急性加重（AE）是特发性肺纤维化（AE-IPF）的严重并发症。 2016年，国际工作组修订了其定义和诊断标准；然而，很少有研究评估其他纤维化间质性肺疾病 (FILD) 患者的 AE 发生频率和预后。方法 我们使用了 2008 年 1 月至 2015 年 7 月期间最初评估的 1019 名连续间质性肺疾病 (ILD) 患者的数据。所有受试者诊断均于 2018 年 12 月通过多学科讨论做出。ILD 被分类为 IPF（n = 462）和其他 FILD，包括非特异性间质性肺炎（n = 22）、慢性过敏性肺炎（n = 29）、结缔组织与疾病相关的 ILD (n = 205) 和无法分类的 ILD (n = 209)。使用 2016 年 AE-IPF 的定义，我们确定了所有患有 AE 的受试者。结果 在观察期间，193 名患者经历了首次 AE（AE-FILD n = 69，AE-IPF n = 124）。 FILD 中出现首次 AE 的时间明显长于 IPF（对数秩检验，P < 0.001）。在调整潜在影响的混杂因素后，与 IPF 相比，FILD 仍然是首次 AE 时间较长的显着预测因子（风险比：0.453；95% CI：0.317-0.647，P = 0.006）。在多变量 Cox 比例分析中，基线疾病严重程度与 AE-ILD 的发生率密切相关。即使在调整其他临床变量后，AE 也会对总生存期产生负面影响。 AE-FILD 和 AE-IPF 显示出类似的不良短期结果。结论 所有形式的 ILD 都有发生 AE 的风险，并且与 AE-IPF 具有相似的结果。
Background and objective Acute exacerbation (AE) is a severe complication of idiopathic pulmonary fibrosis (AE-IPF). In 2016, an international working group revised its definition and diagnostic criteria; however, few studies have assessed the frequency and prognosis of AE in patients with other fibrotic interstitial lung diseases (FILD). Methods We used data from 1019 consecutive interstitial lung disease (ILD) patients initially evaluated between January 2008 and July 2015. All subject diagnoses were made by multidisciplinary discussion in December 2018. ILD was categorized as IPF (n = 462) and other FILD which included non-specific interstitial pneumonia (n = 22), chronic hypersensitivity pneumonitis (n = 29), connective tissue disease-associated ILD (n = 205) and unclassifiable ILD (n = 209). Using the 2016 definition of AE-IPF, we identified all subjects with an AE. Results During the observational period, 193 patients experienced a first AE (AE-FILD n = 69, AE-IPF n = 124). The time to first AE was significantly longer in FILD than IPF (log-rank test, P < 0.001). After adjusting for potentially influential confounders, FILD remained a significant predictor of longer time to first AE compared with IPF (hazard ratio: 0.453; 95% CI: 0.317-0.647, P = 0.006). In a multivariate Cox proportional analysis, baseline disease severity was closely associated with the incidence of AE-ILD. Even after adjustment for other clinical variables, AE had a negative impact on overall survival. AE-FILD and AE-IPF showed similar poor short-term outcomes. Conclusion All forms of ILD are at risk of AE and have a similar outcome to AE-IPF.