Pleuroparenchymal fibroelastosis in association with carcinomas

Respirology. 2016 Jan;21(1):191-4. doi: 10.1111/resp.12654. Epub 2015 Oct 13.

Abstract

PPFE is a rare disease characterized by upper lobe pleural fibrosis and parenchymal fibroelastosis. Its aetiology is considered idiopathic, although possible causative factors have been described. An association of PPFE with solid tumours is unknown and has not been considered previously. We identified six patients with PPFE, four of them with a coexisting malignancy. The case series suggests that PPFE might be an implication of varying factors rather than being an exclusively idiopathic condition.

Keywords: cancer; interstitial lung disease; lung fibrosis; pleuroparenchymal fibroelastosis.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Aged
  • Biopsy
  • Breast Neoplasms* / complications
  • Breast Neoplasms* / pathology
  • Carcinoma, Neuroendocrine* / complications
  • Carcinoma, Neuroendocrine* / pathology
  • Carcinoma, Non-Small-Cell Lung* / complications
  • Carcinoma, Non-Small-Cell Lung* / pathology
  • Diagnosis, Differential
  • Female
  • Humans
  • Lung* / diagnostic imaging
  • Lung* / pathology
  • Male
  • Middle Aged
  • Pancreatic Neoplasms* / complications
  • Pancreatic Neoplasms* / pathology
  • Patient Outcome Assessment
  • Pleural Diseases* / diagnosis
  • Pleural Diseases* / etiology
  • Pleural Diseases* / pathology
  • Pleural Diseases* / physiopathology
  • Pulmonary Fibrosis* / diagnosis
  • Pulmonary Fibrosis* / etiology
  • Pulmonary Fibrosis* / pathology
  • Pulmonary Fibrosis* / physiopathology
  • Respiratory Function Tests / methods
  • Tomography, X-Ray Computed / methods