TY - JOUR
T1 - Pharmacological management of progressive-fibrosing interstitial lung diseases
T2 - A review of the current evidence
AU - Richeldi, Luca
AU - Varone, Francesco
AU - Bergna, Miguel
AU - de Andrade, Joao
AU - Falk, Jeremy
AU - Hallowell, Robert
AU - Jouneau, Stéphane
AU - Kondoh, Yasuhiro
AU - Morrow, Lee
AU - Randerath, Winfried
AU - Strek, Mary
AU - Tabaj, Gabriela
N1 - Funding Information:
The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors. Writing assistance was provided by Tanya Chaudry of GeoMed, an Ashfield company, part of UDG Healthcare plc, which was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI). BIPI was given the opportunity to review the manuscript for medical and scientific accuracy as well as intellectual property considerations.Conflict of interest: L. Richeldi reports grants and personal fees from InterMune, personal fees from Sanofi-Aventis, Roche, ImmuneWorks, Shionogi, Boehringer Ingelheim, Celgene, Nitto, Fibrogen, Promedior, Bristol Myers Squibb and DynaMed, outside the submitted work. F. Varone reports personal fees from Boehringer Ingelheim and Hoffmann-La Roche, outside the submitted work. M. Bergna received research grants from Novartis, GSK, Boehringer Ingelheim, Roche, AstraZeneca and Sanofi, and advisory and investigator fees from GSK, AstraZeneca and Novartis. J. de Andrade reports grants from NIH/NHLBI, grants and personal fees from Boehringer Ingelheim and Genentech, and grants from Fibrogen and GBT, outside the submitted work. J. Falk has nothing to disclose. R. Hallowell has nothing to disclose. S. Jouneau reports grants from AIRB, Boehringer Ingelheim, LVL, Novartis and Roche, and personal fees from Actelion, AIRB, AstraZeneca, BMS, Boehringer Ingelheim, Chiesi, GSK, LVL, Mundipharma, Novartis, Pfizer and Roche, outside the submitted work. Y. Kondoh reports personal lecture fees from Boehringer Ingelheim Co. which is relevant to the presentation work. Y. Kondoh also reports advisory board fees and personal fees from Asahi Kasei Pharma Corp., Ltd, and personal fees from Eisai Inc., Kyorin Pharmaceutical Co., Ltd, Novartis Pharma K.K., Shionogi & Co., Ltd and Teijin Pharma Ltd outside the submitted work. L. Morrow reports grants from Boehringer Ingelheim and Genentech, outside the submitted work. W. Randerath reports nonfinancial support from Boehringer, during the conduct of the study; and grants from Boehringer Ingelheim and Roche Pharma, outside the submitted work. M. Strek reports grants from Boehringer and Roche; and personal fees from Boehringer Ingelheim, outside the submitted work. G. Tabaj has nothing to disclose.
Funding Information:
Acknowledgements: The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors. Writing assistance was provided by Tanya Chaudry of GeoMed, an Ashfield company, part of UDG Healthcare plc, which was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI). BIPI was given the opportunity to review the manuscript for medical and scientific accuracy as well as intellectual property considerations.
Funding Information:
Conflict of interest: L. Richeldi reports grants and personal fees from InterMune, personal fees from Sanofi-Aventis, Roche, ImmuneWorks, Shionogi, Boehringer Ingelheim, Celgene, Nitto, Fibrogen, Promedior, Bristol Myers Squibb and DynaMed, outside the submitted work. F. Varone reports personal fees from Boehringer Ingelheim and Hoffmann-La Roche, outside the submitted work. M. Bergna received research grants from Novartis, GSK, Boehringer Ingelheim, Roche, AstraZeneca and Sanofi, and advisory and investigator fees from GSK, AstraZeneca and Novartis. J. de Andrade reports grants from NIH/NHLBI, grants and personal fees from Boehringer Ingelheim and Genentech, and grants from Fibrogen and GBT, outside the submitted work. J. Falk has nothing to disclose. R. Hallowell has nothing to disclose. S. Jouneau reports grants from AIRB, Boehringer Ingelheim, LVL, Novartis and Roche, and personal fees from Actelion, AIRB, AstraZeneca, BMS, Boehringer Ingelheim, Chiesi, GSK, LVL, Mundipharma, Novartis, Pfizer and Roche, outside the submitted work. Y. Kondoh reports personal lecture fees from Boehringer Ingelheim Co. which is relevant to the presentation work. Y. Kondoh also reports advisory board fees and personal fees from Asahi Kasei Pharma Corp., Ltd, and personal fees from Eisai Inc., Kyorin Pharmaceutical Co., Ltd, Novartis Pharma K.K., Shionogi & Co., Ltd and Teijin Pharma Ltd outside the submitted work. L. Morrow reports grants from Boehringer Ingelheim and Genentech, outside the submitted work. W. Randerath reports nonfinancial support from Boehringer, during the conduct of the study; and grants from Boehringer Ingelheim and Roche Pharma, outside the submitted work. M. Strek reports grants from Boehringer and Roche; and personal fees from Boehringer Ingelheim, outside the submitted work. G. Tabaj has nothing to disclose.
Publisher Copyright:
©ERS 2018.
PY - 2018
Y1 - 2018
N2 - A proportion of patients with interstitial lung diseases (ILDs) are at risk of developing a progressive-fibrosing phenotype, which is associated with a deterioration in lung function and early mortality. In addition to idiopathic pulmonary fibrosis (IPF), fibrosing ILDs that may present a progressive phenotype include idiopathic nonspecific interstitial pneumonia, connective tissue disease-associated ILDs, hypersensitivity pneumonitis, unclassifiable idiopathic interstitial pneumonia, ILDs related to other occupational exposures and sarcoidosis. Corticosteroids and/or immunosuppressive therapies are sometimes prescribed to patients with these diseases. However, this treatment regimen may not be effective, adequate on its own or well tolerated, suggesting that there is a pressing need for efficacious and better tolerated therapies. Currently, the only approved treatments to slow disease progression in patients with IPF are nintedanib and pirfenidone. Similarities in pathobiological mechanisms leading to fibrosis between IPF and other ILDs that may present a progressive-fibrosing phenotype provide a rationale to suggest that nintedanib and pirfenidone may be therapeutic options for patients with the latter diseases. This review provides an overview of the therapeutic options currently available for patients with fibrosing ILDs, including fibrosing ILDs that may present a progressive phenotype, and explores the status of the randomised controlled trials that are underway to determine the efficacy and safety of nintedanib and pirfenidone.
AB - A proportion of patients with interstitial lung diseases (ILDs) are at risk of developing a progressive-fibrosing phenotype, which is associated with a deterioration in lung function and early mortality. In addition to idiopathic pulmonary fibrosis (IPF), fibrosing ILDs that may present a progressive phenotype include idiopathic nonspecific interstitial pneumonia, connective tissue disease-associated ILDs, hypersensitivity pneumonitis, unclassifiable idiopathic interstitial pneumonia, ILDs related to other occupational exposures and sarcoidosis. Corticosteroids and/or immunosuppressive therapies are sometimes prescribed to patients with these diseases. However, this treatment regimen may not be effective, adequate on its own or well tolerated, suggesting that there is a pressing need for efficacious and better tolerated therapies. Currently, the only approved treatments to slow disease progression in patients with IPF are nintedanib and pirfenidone. Similarities in pathobiological mechanisms leading to fibrosis between IPF and other ILDs that may present a progressive-fibrosing phenotype provide a rationale to suggest that nintedanib and pirfenidone may be therapeutic options for patients with the latter diseases. This review provides an overview of the therapeutic options currently available for patients with fibrosing ILDs, including fibrosing ILDs that may present a progressive phenotype, and explores the status of the randomised controlled trials that are underway to determine the efficacy and safety of nintedanib and pirfenidone.
UR - http://www.scopus.com/inward/record.url?scp=85058916693&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85058916693&partnerID=8YFLogxK
U2 - 10.1183/16000617.0074-2018
DO - 10.1183/16000617.0074-2018
M3 - Review article
C2 - 30578333
AN - SCOPUS:85058916693
VL - 27
JO - European Respiratory Review
JF - European Respiratory Review
SN - 0905-9180
IS - 150
M1 - 180074
ER -