Background: In patients with fibrotic interstitial lung disease (ILD), a progressive pulmonary fibrosis (PPF) typically demonstrates worsening respiratory symptoms, lung function decline and continuing fibrosis. The goal of this study was to compare the three different diagnostic criteria of PPF.
Methods: Except for idiopathic pulmonary fibrosis (IPF), all consecutive adult patients with fibrotic ILD were retrospectively examined for the three predefined diagnostic criteria of PPF. The three criteria assessed the disease progression in preceding 6 (0.5-year), 12 (1-year) and 24 (2-year) months respectively. The clinical characteristics, decline in predicted percent of forced vital capacity (FVC%) and survival of three groups were compared, followed by determination of risk factors for mortality.
Results: We identified 246 patients by 0.5-year standard, 154 patients by 1-year standard and 281 patients by 2-year standard. Among them, 95% of patients in 1-year group were also included in 2-year group. The average 1-year decline in FVC% was -1.0%, -2.7%, and -4.1% for 0.5-, 1-, and 2-year group respectively. The 4-year survival rate was 74% in 0.5-year group, 66% in 1-year group, and 62% in 2-year group. In multivariate Cox model, only baseline predicted percent diffusing capacity of the lungs for carbon monoxide (DLCO%) <50% was correlated with mortality, with a hazard ratio of 3.4 (95% CI: 1.1-10.6, P=0.03).
Conclusions: In the current situations, both the 1- and 2-year criterion are the reasonable choice to define PPF both in researches and clinical practice, and DLCO% is an independent predictor for mortality of PPF.
Keywords: Progressive pulmonary fibrosis (PPF); diagnostic criteria; mortality; risk factors.
2024 Journal of Thoracic Disease. All rights reserved.