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Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE).

Hideaki Yamakawa1,2, Tsuneyuki Oda3, Keishi Sugino4

  • 1Department of Respiratory Medicine, Saitama Red Cross Hospital, Saitama 330-8553, Japan.

Journal of Clinical Medicine
|July 13, 2024
PubMed
Summary

Pleuroparenchymal fibroelastosis (PPFE) presents varied progression, making prognosis difficult. This review proposes a precision medicine algorithm for PPFE management, emphasizing physique monitoring and pulmonary rehabilitation.

Keywords:
clinical algorithminterstitial lung diseasepleuroparenchymal fibroelastosis

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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.