Idiopathic Pulmonary Fibrosis

  • Venerino Poletti
  • Antonella Arcadu
  • Sissel Kronborg-White
  • Marco Chilosi


Idiopathic pulmonary fibrosis is a severe disorder mainly affecting lungs. It occurs more frequently in smokers or former smokers with an average age of onset around 65. The first pathogenetic event leading to the complete derangement of lung parenchyma is the alveolar stem cell exhaustion due to intrinsic factors (genetic mutations or polymorphisms) and extrinsic insults (cigarette smoke, pollution). The histopathologic background is Usual Interstitial Pneumonitis. Transbronchial cryobiopsy allows harvesting of lung samples large enough to identify the characteristic of this morphologic pattern. The best strategy to maintain a high diagnostic yield is to biopsy at least two different segments.


Idiopathic pulmonary fibrosis (IPF) Usual Interstitial Pneumonitis (UIP) Patchy fibrosis Fibroblastic foci Honeycomb changes Transbronchial cryobiopsy 


  1. 1.
    Barratt SL, Creamer A, Hayton C, Chaudhuri N. Idiopathic pulmonary fibrosis (IPF): an overview. J Clin Med. 2018;7.CrossRefGoogle Scholar
  2. 2.
    Martinez FJ, Collard HR, Pardo A, Raghu G, Richeldi L, Selman M, Swigris JJ, Taniguchi H, Wells AU. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.CrossRefGoogle Scholar
  3. 3.
    Chiba H, Otsuka M, Takahashi H. Significance of molecular biomarkers in idiopathic pulmonary fibrosis: a mini review. Respir Investig. 2018;56:384–91.CrossRefGoogle Scholar
  4. 4.
    Chilosi M, Carloni A, Rossi A, Poletti V. Premature lung aging and cellular senescence in the pathogenesis of idiopathic pulmonary fibrosis and COPD/emphysema. Transl Res. 2013;162:156–73.CrossRefGoogle Scholar
  5. 5.
    Caliò A, Lever V, Rossi A, et al. Increased frequency of bronchiolar histotypes in lung carcinomas associated with idiopathic pulmonary fibrosis. Histopathology. 2017;71:725–35.CrossRefGoogle Scholar
  6. 6.
    Raghu G, Flaherty KR, Lederer DJ, Lynch DA, Colby TV, et al. Use of molecular classifier to identify usual interstitial pneumonia in conventional transbronchial lung biopsy samples: a prospective validation study. Lancet Respir Med. 2019. In press.Google Scholar
  7. 7.
    Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6:138–53.CrossRefGoogle Scholar
  8. 8.
    Ravaglia C, Tomassetti S, Gurioli C, et al. Features and outcome of familial idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31:28–36.PubMedGoogle Scholar
  9. 9.
    Spagnolo P, Tzouvelekis A, Bonella F. The management of patients with idiopathic pulmonary fibrosis. Front Med (Lausanne). 2018;5:148.CrossRefGoogle Scholar
  10. 10.
    Ravaglia C, Wells AU, Tomassetti S, et al. Transbronchial lung cryobiopsy in diffuse parenchymal lung disease: comparison between biopsy from 1 segment and biopsy from 2 segments—diagnostic yield and complications. Respiration. 2017;93:285–92.CrossRefGoogle Scholar
  11. 11.
    Choi Y, Liu TT, Pankratz DG, Colby TV, et al. Identification of usual interstitial pneumonia pattern using RNA-Seq and machine learning: challenges and solutions. BMC Genomics. 2018;19(Suppl 2):101.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Venerino Poletti
    • 1
    • 2
  • Antonella Arcadu
    • 3
  • Sissel Kronborg-White
    • 2
  • Marco Chilosi
    • 4
    • 5
  1. 1.Department of Diseases of the ThoraxOspedale Morgagni-PierantoniForlìItaly
  2. 2.Department of Respiratory Diseases and AllergyAarhus University HospitalAarhusDenmark
  3. 3.Department of Diseases of the Thorax-Pulmonology UnitAzienda Usl della Romagna, Ospedale GB MorgagniForlìItaly
  4. 4.Department of PathologyP. Pederzoli HospitalPeschiera del GardaItaly
  5. 5.Verona UniversityVeronaItaly

Personalised recommendations