Pleuroparenchymal fibroelastosis in rheumatoid arthritis-associated interstitial lung disease.

ABSTRACT:

Background

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD) featuring dense fibrosis of the visceral pleura and subpleural parenchyma, mostly in the upper lobes. PPFE can present in other ILDs, including rheumatoid arthritis-associated ILD (RA-ILD). The aim of this retrospective study was to investigate the prevalence and clinical implications of coexistent PPFE in RA-ILD.

Methods

Overall, 477 patients with RA-ILD were recruited from two cohorts; their clinical data and HRCT images were analysed. The criteria for diagnosing PPFE were (1) pleural thickening with bilateral subpleural dense fibrosis in the upper lobes, (2) evidence of disease progression, and (3) absence of other identifiable aetiologies.

Results

The median follow-up dura

SUBMITTER: Kang J 

PROVIDER: S-EPMC9161503 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature