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Pleuroparenchymal fibroelastosis in heterogeneous clinical conditions: Clinicopathologic analysis of 7 cases

Yoon Jin Cha1, Joungho Han2, Man Pyo Chung3

  • 1Department of Pathology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.

The Clinical Respiratory Journal
|August 27, 2017
PubMed
Abstract

Insights

Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease with unclear features. This study found PPFE can overlap with other lung conditions and has diverse underlying clinical causes.

Area of Science:

  • Pulmonology
  • Pathology
  • Radiology

Background:

  • Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia.
  • Its clinicopathologic features are not well understood.

Purpose of the Study:

  • To investigate the clinical, radiologic, and pathologic features of patients with histologically confirmed PPFE.

Main Methods:

  • Histologic evaluation of PPFE in seven patients.
  • Analysis of clinical data and chest computed tomography (CT) findings.

Main Results:

  • Patients presented with varied clinical histories, including autoimmune markers, organ transplantation, and infections.
  • Pulmonary function tests showed restrictive patterns.
  • Histology revealed variable PPFE extent with nonspecific findings; radiologically, lesions were diffuse, not solely apical.

Conclusions:

  • PPFE may represent a histologic finding that overlaps with other chronic lung diseases.
  • Diverse underlying clinical conditions can be associated with PPFE.
Keywords:
fibroelastosisinterstitial lung diseasepathologypleural fibrosis

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