title: Idiopathic Pleuroparenchymal Fibroelastosis.
abstract: OBJECTIVE
  Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare fibrosing lung disease, affecting the visceral pleura and the subpleural parenchyma with an upper lobe predilection, included as a distinct clinicopathologic entity in the latest international multidisciplinary classification of the idiopathic interstitial pneumonias (IIP). We aim to summarize the current evidence on IPPFE, in terms of clinical features and potential treatments.
  
  
  RESULTS
  Overall, there is increasing awareness of PPFE in association with a separate ILD pattern. Although an agreed consensus on diagnosis has yet to be defined, a list of radiological and histopathological criteria has been proposed. Due to the unfavorable risk-benefit profile of surgical lung biopsy in a significant proportion of patients, a potential role for transbronchial lung cryobiopsy has been suggested. At present, lung transplantation remains the only curative option.
  
  
  CONCLUSIONS
  The increasing awareness of this condition among specialists has led to more frequent identification of IPPFE. Large international studies are needed to better characterize pathogenesis and pheno/endotypes of disease, a key step towards the development of effective treatments.
authors.affiliation: Department of Biomedical Sciences and Public Health, Università Politecnica delle Marche, Ancona, Italy.|Pulmonary Diseases Unit, Department of Internal Medicine, Azienda Ospedali Riuniti, Ancona, Italy.
authors.forename: Martina
authors.initials: M
authors.lastname: Bonifazi
authors.affiliation: Department of Histopathology, Imperial College, London Royal Brompton and Harefield Hospitals NHS Foundation Trust, London, UK.
authors.forename: M Angeles
authors.initials: MA
authors.lastname: Montero
authors.affiliation: Interstitial Lung Disease Unit, Royal Brompton Hospital, Imperial College, London, UK.
authors.forename: Elisabetta A
authors.initials: EA
authors.lastname: Renzoni
keywords: Clinical features; Diagnosis; Idiopathic interstitial lung disease; Pleuroparenchymal fibroelastosis; Review