Pleuroparenchymal fibroelastosis tied to lower lung function, poorer prognosis in IPF patients

Body mass index (BMI) and lung function are lower in patients with pleuroparenchymal fibroelastosis (PPFE) compared with those without, according to a study. The PPFE arm also has more frequent complications and poorer survival during follow-up.

The authors assessed the clinical significance of the radiologically defined PPFE in patients with idiopathic pulmonary fibrosis (IPF; mean age, 66.4 years; 76.4 percent male) by retrospectively analysing clinical data and chest CT images in 445 participants (biopsy-proven cases, n=165).

The radiological criteria of PPFE was defined as bilateral subpleural dense fibrosis with or without pleural thickening in the upper lobes, evidence of disease progression, and no clinical evidence of identifiable aetiologies.

Of the patients, 28 (6.3 percent) were identified to have PPFE. The median follow-up period was 43.0 months. Those with PPFE demonstrated lower BMI and lung function (ie, forced vital capacity and total lung capacity) at baseline, more frequent pneumothorax and pneumomediastinum, higher decline rates in lung function, and poorer prognosis during follow-up compared with patients without PPFE.

Notably, PPFE was independently associated with pneumothorax or pneumomediastinum (hazard ratio, 2.953, 95 percent confidence interval, 1.350–6.460; p=0.007), but not for mortality in patients with IPF.

“PPFE is characterized by fibrosis in the pleura and subpleural lung parenchyma in the upper lobes, while other types of interstitial lung disease, mainly usual interstitial pneumonia, can be observed in about half of the patients in their lower lobes,” the authors said.