Elisabeth Bendstrup

Cryobiopsies are diagnostic in Pleuroparenchymal and Airway-centered Fibroelastosis

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaperJournal articleResearchpeer-review

  • Sissel Kronborg-White
  • ,
  • Claudia Ravaglia, Ospedale Gb Morgagni
  • ,
  • Alessandra Dubini, Ospedale Gb Morgagni
  • ,
  • Sara Piciucchi, Ospedale Gb Morgagni
  • ,
  • Sara Tomassetti, Ospedale Gb Morgagni
  • ,
  • Elisabeth Bendstrup
  • Venerino Poletti, Ospedale Gb Morgagni

Background: Idiopathic pulmonary fibroelastosis (iPPFE) is a rare lung lesion characterized by pleural and subpleural parenchymal thickening due to accumulation of fibroelastotic tissue. Only recently, a few cases with a peribronchiolar distribution of fibroelastotic tissue have been reported. These lesions are more prominent in the upper lobes. Even though high resolution computed tomography (HRCT) scan features are considered characteristic, a histological confirmation is suggested, mainly when the clinical setting is not clearly defined. However, due to non-negligible complications, a surgical lung biopsy is not often recommended. The prognosis is usually poor and currently, the only effective treatment is lung transplantation. Method: Patients with a multidisciplinary diagnosis of iPPFE or airway-centered fibroelastosis (airway-centered FE), with histological confirmation by transbronchial cryobiopsy, were identified from an ongoing interstitial lung disease registry. Data on patient demographics, HRCT patterns, size and number of biopsies, histology patterns and complications were registered. Results: Seven patients were diagnosed with iPPFE and one patient was diagnosed with airway-centered FE. Pneumothorax was documented in three cases, but none of them required a chest tube. No other complications during or after the procedure were observed. Conclusion: This study suggests that using cryobiopsies in the diagnostics of PPFE and airway-centered FE is safe and effective.

Original languageEnglish
Article number135
JournalRespiratory Research
Publication statusPublished - 13 Jul 2018

    Research areas

  • Airway-centered fibroelastosis, Cryobiopsy, Pleuroparenchymal fibroelastosis

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