An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias

William D. Travis, Ulrich Costabel, David M. Hansell, Talmadge E. King, David A. Lynch, Andrew G. Nicholson, Christopher J. Ryerson, Jay H. Ryu, Moisés Selman, Athol U. Wells, Jurgen Behr, Demosthenes Bouros, Kevin K. Brown, Thomas V. Colby, Harold R. Collard, Carlos Robalo Cordeiro, Vincent Cottin, Bruno Crestani, Marjolein Drent, Rosalind F. DuddenJim Egan, Kevin Flaherty, Cory Hogaboam, Yoshikazu Inoue, Takeshi Johkoh, Dong Soon Kim, Masanori Kitaichi, James Loyd, Fernando J. Martinez, Jeffrey Myers, Shandra Protzko, Ganesh Raghu, Luca Richeldi, Nicola Sverzellati, Jeffrey Swigris, Dominique Valeyre

Research output: Contribution to journalArticlepeer-review

1899 Scopus citations


Background: In 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs)definedseven specific entities, andprovided standardized terminology anddiagnosticcriteria. Inaddition, thehistorical "gold standard" of histologic diagnosiswas replaced by amultidisciplinary approach. Since 2002 many publications have provided newinformation about IIPs. Purpose: The objective of this statement is to update the 2002 ATS/ ERS classification of IIPs. Methods: An international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011. Results: Substantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis-interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia is recognized to be heterogeneous. Acute exacerbation of IIPs is now well defined. A substantial percentage of patients with IIP are difficult to classify, often due to mixed patterns of lung injury. A classification based on observed disease behavior is proposed for patients who are difficult to classify or for entities with heterogeneity in clinical course. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histologic patterns, is introduced. The rapidly evolving field of molecular markers is reviewed with the intent of promoting additional investigations that may help in determining diagnosis, and potentially prognosis and treatment. Conclusions: This update is a supplement to the previous 2002 IIP classification document. It outlines advances in the past decade and potential areas for future investigation.

Original languageEnglish (US)
Pages (from-to)733-748
Number of pages16
JournalAmerican journal of respiratory and critical care medicine
Issue number6
StatePublished - Sep 15 2013


  • Acute interstitial pneumonia
  • Cryptogenic organizing pneumonia
  • Desquamative interstitial pneumonia
  • Idiopathic interstitial pneumonia
  • Nonspecific interstitial pneumonia
  • Respiratory bronchiolitis
  • Usual interstitial pneumonia

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


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