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Review
. 2018 Dec 21;27(150):180074.
doi: 10.1183/16000617.0074-2018. Print 2018 Dec 31.

Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence

Luca Richeldi  1 , Francesco Varone  2 , Miguel Bergna  3 , Joao de Andrade  4 , Jeremy Falk  5 , Robert Hallowell  6 , Stéphane Jouneau  7 , Yasuhiro Kondoh  8 , Lee Morrow  9 , Winfried Randerath  10 , Mary Strek  11 , Gabriela Tabaj  12
Affiliations
Review

Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence

Luca Richeldi et al. Eur Respir Rev. .

Abstract

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.

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Conflict of interest statement

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. Conflict of interest: F. Varone reports personal fees from Boehringer Ingelheim and Hoffmann-La Roche, outside the submitted work. Conflict of interest: M. Bergna received research grants from Novartis, GSK, Boehringer Ingelheim, Roche, AstraZeneca and Sanofi, and advisory and investigator fees from GSK, AstraZeneca and Novartis. Conflict of interest: 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. Conflict of interest: J. Falk has nothing to disclose. Conflict of interest: R. Hallowell has nothing to disclose. Conflict of interest: 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. Conflict of interest: 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. Conflict of interest: L. Morrow reports grants from Boehringer Ingelheim and Genentech, outside the submitted work. Conflict of interest: 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. Conflict of interest: M. Strek reports grants from Boehringer and Roche; and personal fees from Boehringer Ingelheim, outside the submitted work. Conflict of interest: G. Tabaj has nothing to disclose.

Figures

FIGURE 1
FIGURE 1
Annual rate of change from baseline over time in forced vital capacity (FVC) in the a) phase II TOMORROW [12], b) phase III INPULSIS-1 [13] and c) INPULSIS-2 [13] studies. #: p=0.01; ***: p<0.001.
FIGURE 2
FIGURE 2
Mean change from baseline in percentage predicted forced vital capacity (FVC) in the a) phase III CAPACITY [27] and b) ASCEND [14] studies. #: n=174; : n=173 +: n=171; §: n=277; ƒ: n=278. Reproduced from [14] with permission.
See this image and copyright information in PMC

Comment in

  • doi: 10.1183/16000617.0110-2018

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