Is unclassifiable idiopathic interstitial pneumonia a result of idiopathic pleuroparenchymal fibroelastosis?

Respir Investig. 2020 Nov;58(6):502-505. doi: 10.1016/j.resinv.2020.03.003. Epub 2020 Apr 8.

Abstract

The present article reports the case of a 51-year-old female patient who was clinically diagnosed with idiopathic pleuroparenchymal fibroelastosis in the initial phase of the disease. The upper and lower lobes showed a pleuroparenchymal fibroelastosis pattern and reticular shadow, respectively. Both upper and lower shadows gradually progressed. Five years following the first detection of the chest shadow, left single-lung transplantation was performed. In the multidisciplinary discussion held after the lung transplantation, most participants recognized this end-stage lung disease as unclassifiable idiopathic interstitial pneumonia though the major pathological findings showing a pleuroparenchymal fibroelastosis pattern.

Keywords: Lung transplantation; Multidisciplinary discussion; Pleuroparenchymal fibroelastosis; Unclassifiable idiopathic interstitial pneumonia.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Hamman-Rich Syndrome*
  • Humans
  • Idiopathic Interstitial Pneumonias*
  • Lung / diagnostic imaging
  • Lung Diseases, Interstitial* / diagnostic imaging
  • Lung Diseases, Interstitial* / etiology
  • Lung Transplantation*
  • Middle Aged