Unclassifiable interstitial lung disease: from phenotyping to possible treatments

Current Opinion in Pulmonary Medicine
Sabina A Guler, Christopher J Ryerson

Abstract

Accurate diagnosis of interstitial lung diseases (ILDs) can be challenging, and a substantial percentage of ILD patients remain unclassifiable even after thorough assessment by an experienced multidisciplinary team. In this review, we summarize the recent literature on the definition, prevalence, diagnosis, treatment, and prognosis of unclassifiable ILD, and also discuss important current issues and provide future perspectives on the classification of ILD. Approximately 12% of patients with ILD are considered unclassifiable, with large variability across previous studies that is in part secondary to inconsistent definitions of unclassifiable ILD and other ILD subtypes. A recent International Working Group suggested that unclassifiable ILD should be defined by the absence of a leading diagnosis that is considered more likely than not after multidisciplinary discussion of all available information. Clinical features and outcomes of unclassifiable ILD are intermediate between idiopathic pulmonary fibrosis and nonidiopathic pulmonary fibrosis ILD cohorts, and choices for pharmacotherapy should be considered on a case-by-case basis. Recent studies have provided additional data on the clinical features and prognosis of unclassifiable...Continue Reading

References

Mar 5, 2003·American Journal of Respiratory and Critical Care Medicine·Kevin R FlahertyFernando J Martinez
Jul 5, 2003·American Journal of Respiratory and Critical Care Medicine·Yves LacasseUNKNOWN HP Study Group
Apr 13, 2004·European Journal of Epidemiology·José Luis López-CamposUNKNOWN Registry of Interstitial Lung Diseases
Jun 12, 2004·Respiratory Medicine·Michiel J ThomeerMaurits Demedts
Jul 17, 2004·American Journal of Respiratory and Critical Care Medicine·Kevin R FlahertyFernando J Martinez
Sep 17, 2005·American Journal of Respiratory and Critical Care Medicine·Moises SelmanAlbert Zlotnik
Apr 8, 2011·American Journal of Respiratory and Critical Care Medicine·Ganesh RaghuUNKNOWN ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis
Jan 3, 2012·Respiratory Medicine·Atsuko HaraShigeru Kohno
May 23, 2012·The New England Journal of Medicine·UNKNOWN Idiopathic Pulmonary Fibrosis Clinical Research NetworkFernando J Martinez
Dec 12, 2012·The European Respiratory Journal·Christopher J RyersonHarold R Collard
Sep 4, 2013·The European Respiratory Journal·Vincent Cottin, Athol Wells
Sep 17, 2013·American Journal of Respiratory and Critical Care Medicine·William D TravisUNKNOWN ATS/ERS Committee on Idiopathic Interstitial Pneumonias
Oct 12, 2013·Chest·Christopher J RyersonHarold R Collard
Nov 30, 2013·Respirology : Official Journal of the Asian Pacific Society of Respirology·Gregory J KeirElisabetta A Renzoni
Apr 22, 2014·Translational Research : the Journal of Laboratory and Clinical Medicine·Ivana V Yang, David A Schwartz
May 3, 2014·The European Respiratory Journal·Lauren TroyTamera Corte
May 20, 2014·The New England Journal of Medicine·Luca RicheldiUNKNOWN INPULSIS Trial Investigators
May 20, 2014·The New England Journal of Medicine·Talmadge E KingUNKNOWN ASCEND Study Group
May 13, 2015·Journal of Medical Imaging and Radiation Oncology·Miranda L SiemienowiczRobert P J Fabiny
Jul 15, 2015·The European Respiratory Journal·Aryeh FischerUNKNOWN “ERS/ATS Task Force on Undifferentiated Forms of CTD-ILD”
Aug 5, 2015·American Journal of Respiratory and Critical Care Medicine·Daina B EspositoTrung N Tran
Nov 13, 2015·American Journal of Respiratory and Critical Care Medicine·Sara TomassettiVenerino Poletti
Nov 17, 2015·American Journal of Respiratory and Critical Care Medicine·Brett Ley, Harold R Collard
Dec 10, 2015·American Journal of Respiratory and Critical Care Medicine·John P HutchinsonRichard B Hubbard
Apr 8, 2016·Journal of Proteome Research·Yun Pyo KangSung-Woo Park
Jun 23, 2016·American Journal of Respiratory and Critical Care Medicine·Ganesh RaghuDavid M Hansell
Nov 2, 2016·The European Respiratory Journal·John P HutchinsonRichard B Hubbard
Nov 20, 2016·Respirology : Official Journal of the Asian Pacific Society of Respirology·Hadeel Khadawardi, Marco Mura
Nov 21, 2016·Chest·Karen C PattersonWallace T Miller
Mar 16, 2017·Respirology : Official Journal of the Asian Pacific Society of Respirology·Charlotte HyldgaardOle Hilberg
Mar 30, 2017·Annals of the American Thoracic Society·Brett LeyHarold R Collard
Apr 18, 2017·American Journal of Respiratory and Critical Care Medicine·Christopher J RyersonHarold R Collard
Nov 21, 2017·The Lancet. Respiratory Medicine·David A LynchAthol U Wells
May 22, 2018·Annals of the American Thoracic Society·Sabina A GulerChristopher J Ryerson

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Citations

Jul 1, 2020·Therapeutic Advances in Respiratory Disease·Noriyuki EnomotoTakafumi Suda
Mar 19, 2019·The European Respiratory Journal·Tomoyuki FujisawaTakafumi Suda
May 19, 2021·QJM : Monthly Journal of the Association of Physicians·R SahaJ D Dodd
Jun 27, 2021·Current Opinion in Pulmonary Medicine·Marissa O'CallaghanCormac McCarthy

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