Macmurdo Maeve G, Ji Xinge, Pimple Pratik, Olson Amy L, Milinovich Alex, Martyn-Dow Blaine, Pande Aman, Zajichek Alex, Bauman Janine, Bender Shaun, Conoscenti Craig, Sugano David, Kattan Michael W, Culver Daniel A
Respiratory Institute, Cleveland Clinic, United States.
Department of Quantitative Health Sciences, Cleveland Clinic, United States.
Respir Med. 2024 Jun;227:107656. doi: 10.1016/j.rmed.2024.107656. Epub 2024 Apr 30.
The proportion of patients who develop progressive pulmonary fibrosis (PPF), along with risk factors for progression remain poorly understood.
To examine factors associated with an increased risk of developing PPF among patients at a referral center.
We identified patients with a diagnosis of interstitial lung disease (ILD) seen within the Cleveland Clinic Health System. Utilizing a retrospective observational approach we estimated the risk of developing progression by diagnosis group and identified key clinical predictors using the FVC component of both the original progressive fibrotic interstitial lung disease (PFILD) and the proposed PPF (ATS) criteria.
We identified 5934 patients with a diagnosis of ILD. The cumulative incidence of progression over the 24 months was similar when assessed with the PFILD and PPF criteria (33.1 % and 37.9 % respectively). Of those who met the ATS criteria, 9.5 % did not meet the PFILD criteria. Conversely, 4.3 % of patients who met PFILD thresholds did not achieve the 5 % absolute FVC decline criteria. Significant differences in the rate of progression were seen based on underlying diagnosis. Steroid therapy (HR 1.46, CI 1.31-1.62) was associated with an increased risk of progressive fibrosis by both PFILD and PPF criteria.
Regardless of the definition used, the cumulative incidence of progressive disease is high in patients with ILD in the 24 months following diagnosis. Some differences are seen in the risk of progression when assessed by PFILD and PPF criteria. Further work is needed to identify modifiable risk factors for the development of progressive fibrosis.
发生进行性肺纤维化（PPF）的患者比例以及疾病进展的风险因素仍未得到充分了解。
研究转诊中心患者中发生PPF风险增加的相关因素。
我们在克利夫兰诊所医疗系统中确定了诊断为间质性肺疾病（ILD）的患者。采用回顾性观察方法，我们按诊断组估计疾病进展风险，并使用原始进行性纤维化间质性肺疾病（PFILD）和提议的PPF（美国胸科学会）标准中的FVC成分确定关键临床预测因素。
我们确定了5934例诊断为ILD的患者。使用PFILD和PPF标准评估时，24个月内疾病进展的累积发生率相似（分别为33.1%和37.9%）。符合美国胸科学会标准的患者中，9.5%不符合PFILD标准。相反，符合PFILD阈值的患者中有4.3%未达到FVC绝对值下降5%的标准。根据潜在诊断，疾病进展率存在显著差异。根据PFILD和PPF标准，类固醇治疗（风险比1.46，可信区间1.31 - 1.62）与进行性纤维化风险增加相关。
无论使用何种定义，ILD患者在诊断后的24个月内，进行性疾病的累积发生率都很高。使用PFILD和PPF标准评估时，疾病进展风险存在一些差异。需要进一步开展工作以确定进行性纤维化发展的可改变风险因素。
