Prognostic Impact of Radiologic and Pathologic Features on the Development of Progressive Pulmonary Fibrosis in...

To evaluate the prognostic impact of radiologic and pathologic features in patients with interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis (IPF), and to identify the factors associated with the development of progressive pulmonary fibrosis (PPF) and survival.

This study retrospectively enrolled 75 patients diagnosed with ILD other than IPF who underwent surgical lung biopsy between January 2004 and December 2020. Three chest radiologists independently reviewed the CT features and extent of fibrosis on preoperative and follow-up CT scans. Two pathologists reviewed the histopathological features, including the presence of interstitial pneumonia. The time to PPF and overall survival were estimated using the Kaplan-Meier method. The associations of CT and pathological features with PPF and all-cause mortality were examined using standard Cox regression and time-dependent Cox models, respectively.

A total of 75 non-IPF ILD patients (mean age ± standard deviations 56.4 ± 13.2 years; range, 40-88 years) were enrolled. The median follow-up duration was 75.3 months (range, 7.8-189.8 months). Traction bronchiectasis on CT (adjusted hazard ratio [HR], 6.40; P = 0.003) and body mass index (adjusted HR per 1-kg/m² increase, 0.82; P = 0.002) were found to be significantly associated with PPF in multivariable analysis. Radiological progression (adjusted HR, 18.44; P < 0.001), symptomatic progression (adjusted HR, 4.19; P = 0.011), and age (adjusted HR for 1-year increase, 1.12; P < 0.001) were significantly associated with death.

Traction bronchiectasis on CT was a significant predictor of PPF, while radiologic and symptomatic progression and older age were associated with poorer survival in patients with ILD other than IPF. These findings indicate that careful radiological evaluation and symptom monitoring may help to predict disease progression and outcomes in patients with non-IPF ILD.