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

Yoon Jin Cha, Joungho Han, Man Pyo Chung, Tae Jung Kim, Sumin Shin

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8 Scopus citations


Background and Aims: Pleuroparenchymal fibroelastosis (PPFE) is classified as a rare idiopathic interstitial pneumonia, and its clinicopathologic features remain unclear. We sought to investigate the clinical, radiologic, and pathologic features of patients with histologically proven PPFE. Methods: Seven patients who had histologic features of PPFE that were definitive or consistent with PPFE were included. Clinical data and radiologic findings of chest computed tomography were obtained. Histologic evaluation was performed by 2 pathologists. Results: Four patients had elevated serum autoantibodies, 3 had a history of organ transplantation, and 1 had acute Aspergillus infection. All patients had various degrees of restrictive patterns on pulmonary function tests. Histologic examination revealed a variable extent of PPFE. Fibroblastic foci, patchy lymphocytic infiltration, and pneumothorax were observed, but these findings were nonspecific. Radiologically, PPFE lesions were distributed diffusely, rather than being limited to the upper lungs. Conclusions: PPFE seems to be a histologic finding that may partly overlap with other chronic lung diseases. There can be various underlying clinical states.

Original languageEnglish
Pages (from-to)1495-1502
Number of pages8
JournalClinical Respiratory Journal
Issue number4
StatePublished - Apr 2018

Bibliographical note

Publisher Copyright:
© 2017 John Wiley & Sons Ltd


  • fibroelastosis
  • interstitial lung disease
  • pathology
  • pleural fibrosis


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