Fibrosing interstitial lung diseases: knowns and unknowns

European Respiratory Review : an Official Journal of the European Respiratory Society
Vincent CottinSergio Harari

Abstract

Patients with certain types of fibrosing interstitial lung disease (ILD) are at risk of developing a progressive phenotype characterised by self-sustaining fibrosis, decline in lung function, worsening quality of life, and early mortality. It has been proposed that such progressive fibrosing ILDs, which show commonalities in clinical behaviour and in the pathogenetic mechanisms that drive progressive fibrosis, may be "lumped" together for the purposes of clinical research and, potentially, for treatment. At present, no drugs are approved for the treatment of ILDs other than nintedanib and pirfenidone for the treatment of idiopathic pulmonary fibrosis. For other progressive fibrosing ILDs, the mainstay of drug therapy is immunosuppression. However, it is postulated that, once the response to lung injury in fibrosing ILDs has reached the stage at which fibrosis has become progressive and self-sustaining, targeted antifibrotic therapy would be required to slow disease progression. Nintedanib, an intracellular inhibitor of tyrosine kinases, has shown antifibrotic, anti-inflammatory and vascular remodelling effects in several non-clinical models of fibrosis, irrespective of the trigger for the injury. Ongoing clinical trials will pr...Continue Reading

References

Jan 11, 2003·Arthritis and Rheumatism·I MarieH Courtois
May 4, 2004·The American Journal of Medicine·Jason S VourlekisKevin K Brown
Jun 23, 2006·The New England Journal of Medicine·Donald P TashkinUNKNOWN Scleroderma Lung Study Research Group
Feb 28, 2007·Toxicologic Pathology·Nasreen KhalilRobert O'Connor
Mar 29, 2008·American Journal of Respiratory and Critical Care Medicine·Nicole S L GohAthol U Wells
Jan 17, 2009·Annals of the Rheumatic Diseases·O Kowal-BieleckaUNKNOWN EUSTAR Co-Authors
Sep 21, 2010·American Journal of Respiratory and Critical Care Medicine·Amy L OlsonKevin K Brown
Apr 8, 2011·American Journal of Respiratory and Critical Care Medicine·Ganesh RaghuUNKNOWN ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis
Sep 3, 2011·The European Respiratory Journal·J L HookD J Lederer
Sep 24, 2011·American Journal of Respiratory and Critical Care Medicine·Roland M du BoisAthol U Wells
Oct 14, 2011·The New England Journal of Medicine·Luca RicheldiRoland M du Bois
Jun 9, 2012·American Journal of Respiratory and Critical Care Medicine·Moisés SelmanTalmadge E King
Dec 12, 2012·The European Respiratory Journal·Christopher J RyersonHarold R Collard
Mar 5, 2013·European Respiratory Review : an Official Journal of the European Respiratory Society·Joshua J SolomonGanesh Raghu
Aug 21, 2013·Annals of the American Thoracic Society·Karen C Patterson, Mary E Strek
Sep 17, 2013·American Journal of Respiratory and Critical Care Medicine·William D TravisUNKNOWN ATS/ERS Committee on Idiopathic Interstitial Pneumonias
Jan 1, 2014·Respirology : Official Journal of the Asian Pacific Society of Respirology·Deborah AssayagChristopher J Ryerson
Feb 22, 2014·The Journal of Pharmacology and Experimental Therapeutics·Lutz WollinBernhard Ryffel
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
Aug 28, 2014·American Journal of Respiratory and Critical Care Medicine·Motoki NatsuizakaHiroki Takahashi
Mar 7, 2015·The European Respiratory Journal·Lutz WollinMartin Kolb
Apr 11, 2015·Annals of the Rheumatic Diseases·Jingang HuangJörg H W Distler
Jun 2, 2015·European Respiratory Review : an Official Journal of the European Respiratory Society·Jean-Christophe LegaVincent Cottin
Jul 15, 2015·The European Respiratory Journal·Aryeh FischerUNKNOWN “ERS/ATS Task Force on Undifferentiated Forms of CTD-ILD”
Jul 16, 2015·American Journal of Respiratory and Critical Care Medicine·Ganesh RaghuUNKNOWN Latin American Thoracic Association
Nov 14, 2015·Respirology : Official Journal of the Asian Pacific Society of Respirology·Elizabeth A BelloliKevin R Flaherty
Nov 21, 2015·The European Respiratory Journal·Joshua J SolomonJeffrey J Swigris
Mar 19, 2016·American Journal of Respiratory and Critical Care Medicine·Robert BrownellHarold R Collard
Apr 19, 2016·American Journal of Respiratory and Critical Care Medicine·Anne-Marie RussellToby M Maher
Apr 23, 2016·The European Respiratory Journal·Justin M OldhamMary E Strek
Jun 15, 2016·American Journal of Respiratory and Critical Care Medicine·Harold R CollardFernando J Martinez
Oct 28, 2016·Arthritis & Rheumatology·Jorge A Zamora-LegoffEric L Matteson

❮ Previous
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Citations

Mar 29, 2020·The European Respiratory Journal·Kevin K BrownAthol U Wells
Jul 14, 2020·Current Opinion in Pulmonary Medicine·Amornpun WongkarnjanaMartin R J Kolb
Sep 3, 2020·The New England Journal of Medicine·Marlies Wijsenbeek, Vincent Cottin
Oct 4, 2019·European Respiratory Review : an Official Journal of the European Respiratory Society·Vincent Cottin
May 28, 2019·The European Respiratory Journal·Vincent CottinUNKNOWN Ariane-IPF Clinical Research Collaboration consortium
Jun 23, 2020·Expert Review of Respiratory Medicine·Paroma SarkarNazia Chaudhuri
Jan 31, 2020·Respiratory Research·Alyson W WongSabina A Guler
Jul 10, 2019·The European Respiratory Journal·Lutz WollinMartin Kolb
Nov 12, 2019·Clinical & Translational Immunology·Andrew McLean-TookeFiona Lake
Oct 1, 2019·The New England Journal of Medicine·Kevin R FlahertyUNKNOWN INBUILD Trial Investigators
May 28, 2020·International Journal of Molecular Sciences·Wiwin Is EffendiResti Yudhawati
Oct 18, 2020·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Pan ChenPeibo Li
Jun 23, 2020·American Journal of Respiratory and Critical Care Medicine·Gillian C GoobieKerri A Johannson
Jan 1, 2021·International Journal of Molecular Sciences·Anna LöfdahlGunilla Westergren-Thorsson
May 25, 2021·Clinics in Chest Medicine·Bridget F Collins, Fabrizio Luppi
Jun 18, 2021·The Nurse Practitioner·Tyonn BarberaNina M Patel
Jul 18, 2021·Patient Education and Counseling·Joanna Y T LeeAnne E Holland
Aug 7, 2021·Clinics in Chest Medicine·Theodoros KarampitsakosArgyris Tzouvelekis
Aug 12, 2021·Therapeutic Advances in Musculoskeletal Disease·Masataka KuwanaAlbert Selva-O'Callaghan
Aug 17, 2021·Frontiers in Medicine·Lurdes Planas-CerezalesMaría Molina-Molina
Jul 6, 2021·Current Opinion in Pulmonary Medicine·Anand DevarajNicola Sverzellati
Aug 17, 2021·Current Opinion in Pulmonary Medicine·Claudia Ravaglia, Andrew G Nicholson
Sep 4, 2021·Therapeutic Advances in Respiratory Disease·Anoop M NambiarJeffrey A Sparks
May 22, 2020·La Presse médicale·Vincent Cottin, Claudia Valenzuela

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