title: Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy.
abstract: OBJECTIVE
  Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) with variable clinical and radiological features. Diagnosis is based on histology obtained by surgical lung biopsy, which is associated with significant mortality and morbidity. This study aims to briefly review PPFE and discuss the role of CT-guided transthoracic core lung biopsy (TTB) in its diagnosis.
  
  
  METHODS
  Four cases of PPFE diagnosed at our institution with TTB are reported and discussed.
  
  
  RESULTS
  Clinical, radiological and histological features are in agreement with the previous literature cases. TTB provided the diagnosis in all cases. Iatrogenic pneumothorax was the main complication in all patients. Placement of a chest tube was needed in three patients. An overlap between PPFE and other interstitial lung diseases (ILD) was documented.
  
  
  CONCLUSIONS
  PPFE is an underdiagnosed IIP, so radiologist awareness of it needs to be widespread in patients with fibrosis with apical-caudal distribution. Coexistence of different lung diseases strengthens the idea of a predisposing factor. TTB proved to be a good diagnostic tool and can be considered the first choice for invasive assessment of these patients. PFFE has a variable course with no established therapeutic options; therefore a multidisciplinary team is crucial in the approach to patients with ILD.
  • PPFE should be considered in the differential diagnosis of fibrosis with apical-caudal distribution. • CT-guided TTB can be considered the first choice for invasive assessment of PPFE. • Site of biopsy has to be chosen carefully in order not to miss PPFE. • Coexistence of different lung diseases strengthens the idea of a predisposing factor. • A multidisciplinary team is crucial in the approach to patients with ILD.
authors.affiliation: Department of Radiology, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal. catiamcesteves@gmail.com.
authors.forename: Cátia
authors.initials: C
authors.lastname: Esteves
authors.affiliation: Department of Radiology, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal.
authors.forename: Francisco R
authors.initials: FR
authors.lastname: Costa
authors.affiliation: Department of Pneumology, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal.
authors.forename: Margarida T
authors.initials: MT
authors.lastname: Redondo
authors.affiliation: Department of Pathology, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal.|Faculty of Medicine, University of Porto, Porto, Portugal.
authors.forename: Conceição S
authors.initials: CS
authors.lastname: Moura
authors.affiliation: Department of Pathology, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal.
authors.forename: Susana
authors.initials: S
authors.lastname: Guimarães
authors.affiliation: Department of Pneumology, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal.|Faculty of Medicine, University of Porto, Porto, Portugal.
authors.forename: António
authors.initials: A
authors.lastname: Morais
authors.affiliation: Department of Radiology, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal.|Faculty of Medicine, University of Porto, Porto, Portugal.
authors.forename: José M
authors.initials: JM
authors.lastname: Pereira
keywords: Biopsy; Computed tomography; Fibrosis; Idiopathic interstitial pneumonia; Lung