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Idiopathic pleuroparenchymal fibroelastosis - A rare idiopathic interstitial pneumonia

Balamugesh Thangakunam1, Barney T J Isaac1, Devasahayam Jesudas Christopher1

  • 1Department of Pulmonary Medicine, Christian Medical College, Vellore, India.

Respiratory Medicine Case Reports
|May 4, 2016
PubMed

Insights

Idiopathic pleuroparenchymal fibroelastosis, a rare lung disease, is increasingly recognized. This report details the first case from India, contributing to understanding this interstitial pneumonia.

Area of Science:

  • Pulmonology
  • Respiratory Medicine
  • Interstitial Lung Diseases

Background:

  • Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare idiopathic interstitial pneumonia.
  • First described in 2004, IPPFE was incorporated into the ATS/ERS classification in 2013.
  • Limited case reports highlight the emerging diagnostic criteria and debated etiology of IPPFE.

Observation:

  • This study presents a case of pleuroparenchymal fibroelastosis in India.
  • The case is likely idiopathic, representing the first reported instance from the region.
  • The report includes a concise review of existing literature on IPPFE.

Findings:

  • The case contributes to the scarce global data on idiopathic pleuroparenchymal fibroelastosis.
  • This report underscores the need for further research into the diagnosis and causes of IPPFE.
  • The presentation highlights the geographical diversity of IPPFE cases.

Implications:

  • This case report expands the geographical understanding of idiopathic pleuroparenchymal fibroelastosis.
  • It emphasizes the importance of recognizing IPPFE in diverse clinical settings.
  • Further research is crucial for refining diagnostic criteria and elucidating the etiology of IPPFE.
Keywords:
Idiopathic interstitial pneumoniaInterstitial lung diseasePleuroparenchymal fibroelastosis

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