Abstract
BackgroundClinical course of pleuroparenchymal fibroelastosis (PPFE) shows considerable variation among patients, but there is no established prognostic prediction model for PPFE.MethodsThe prediction model was developed using retrospective data from two cohorts: our single-center cohort and a nationwide multicenter cohort involving 21 institutions. Cox regression analyses were used to identify prognostic factors. The total score was defined as the weighted sum of values for the selected variables. The performance of the prediction models was evaluated by Harrell’s concordance index (C-index). We also examined the usefulness of the gender-age-physiology (GAP) model for predicting the prognosis of PPFE patients.ResultsWe examined 104 patients with PPFE (52 cases from each cohort). In a multivariate Cox analysis, a lower forced vital capacity (FVC [defined as FVC < 65%]; hazard ratio [HR], 2.23), a history of pneumothorax (HR, 3.27), the presence of a lower lobe interstitial lung disease (ILD) (HR, 2.31), and higher serum Krebs von den Lungen-6 (KL-6) levels (> 550 U/mL, HR, 2.56) were significantly associated with a poor prognosis. The total score was calculated as 1 × (FVC, < 65%) + 1 × (history of pneumothorax) + 1 × (presence of lower lobe ILD) + 1 × (KL-6, > 550 U/mL). PPFE patients were divided into three groups based on the prognostic score: stage I (0–1 points), stage II (2 points), and stage III (3–4 points). The survival rates were significantly different in each stage. The GAP stage was significantly associated with the prognosis of PPFE, but no difference was found between moderate (stage II) and severe (stage III) disease. Our new model for PPFE patients (PPFE Prognosis Score) showed better performance in the prediction of mortality in comparison to the GAP model (C-index of 0.713 vs. 0.649).ConclusionsOur new model for PPFE patients could be useful for predicting their prognosis.
Highlights
Clinical course of pleuroparenchymal fibroelastosis (PPFE) shows considerable variation among patients, but there is no established prognostic prediction model for PPFE
Pleuroparenchymal fibroelastosis (PPFE) is a rare subtype of interstitial lung disease (ILD) that consists of elastofibrosis that is predominantly located in the upper lobes [1,2,3,4,5]
We examined the clinical background, including age, sex, underlying diseases, smoking history, history of pneumothorax before the diagnosis, and body mass index (BMI); the physical examination of fine crackles and finger clubbing; laboratory findings (KL-6, surfactant protein A [SP-A], and SP-D); respiratory function parameters (FVC, the ratio of residual volume (RV) to total lung capacity [RV/TLC], and DLco); the six-minute walk distance, and the lowest SpO2
Summary
Clinical course of pleuroparenchymal fibroelastosis (PPFE) shows considerable variation among patients, but there is no established prognostic prediction model for PPFE. Pleuroparenchymal fibroelastosis (PPFE) is a rare subtype of interstitial lung disease (ILD) that consists of elastofibrosis that is predominantly located in the upper lobes [1,2,3,4,5]. The gender-age-physiology (GAP) model has been widely used as a prognostic scoring system for patients with idiopathic pulmonary fibrosis (IPF) [23]. This model is reported to be useful for predicting mortality in patients with chronic hypersensitivity pneumonitis, connective tissue disease-associated ILD, idiopathic nonspecific interstitial pneumonia, and unclassifiable ILD [24]. Shioya et al reported that a higher GAP model was associated with poorer survival in a small retrospective cohort of patients with idiopathic PPFE [25].
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