Idiopathic pleuroparenchymal fibroelastosis: diagnosis and management

Abstract

Introduction: Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare progressive interstitial lung disease characterized by upper-lobe fibrosis, severe restrictive impairment, and poor prognosis. Unlike idiopathic pulmonary fibrosis, in which acute exacerbations, chronic respiratory failure, and lung cancer are the major causes of death, iPPFE primarily leads to progressive respiratory failure, often complicated by malnutrition and recurrent pneumothorax. Despite growing recognition, its pathogenesis remains unclear and no effective treatments exist. Areas covered: This review summarizes the epidemiological, clinical, radiological, and pathological features of iPPFE, as well as diagnostic and prognostic advancements. Key prognostic factors include weight loss, reduced forced vital capacity, hypercapnia, and lower-lobe interstitial pneumonia. Serum biomarkers (e.g. latent transforming growth factor-beta binding protein-4) are being explored for early detection and prognostic purposes. Although antifibrotic agents show limited efficacy, supportive care–pulmonary rehabilitation, nutritional management, and pneumothorax prevention–remains essential. Research on the fibroelastotic pathways may inform the development of future therapies. Expert opinion: IPPFE remains a challenging disease. Therefore, early diagnosis and comprehensive management of this condition are crucial. Future research should refine prognostic models and explore novel therapeutic approaches for treating fibroelastosis. Lung transplantation may be an option for select patients. Further studies are required to optimize these outcomes.