Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE)

Area of Science:

• Pulmonology
• Respiratory Medicine
• Fibrotic Lung Diseases

Background:

• Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease characterized by fibrosis of the pleura and subpleural parenchyma, primarily affecting upper lobes.
• PPFE exhibits heterogeneous etiologies and disease progression, complicating accurate prognosis and treatment strategies.
• Current management lacks a standardized algorithm, highlighting the need for evidence-based approaches.

Purpose of the Study:

• To review the characteristics of Pleuroparenchymal fibroelastosis (PPFE) based on existing literature.
• To propose a precision medicine algorithm for the management of PPFE patients.
• To emphasize the importance of physique monitoring alongside pulmonary function and radiological evaluation.

Main Methods:

• Comprehensive review of previous studies on Pleuroparenchymal fibroelastosis (PPFE).
• Analysis of disease characteristics, progression patterns, and etiological factors.
• Development of a proposed management algorithm integrating current evidence.

Main Results:

• Pleuroparenchymal fibroelastosis (PPFE) prognosis is challenging due to heterogeneous disease courses.
• "Uncoordinated breathing" is hypothesized as a primary cause of dyspnea in PPFE patients.
• Physique monitoring, nutritional care, and pulmonary rehabilitation are crucial components for PPFE management.

Conclusions:

• A standardized clinical algorithm is essential for accurate Pleuroparenchymal fibroelastosis (PPFE) assessment and management.
• Precision medicine approach, incorporating physique maintenance, is proposed for PPFE.
• Multidisciplinary pulmonologist input is required to refine and implement the proposed PPFE management algorithm.