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.
| Original language | English (US) |
|---|---|
| Article number | 180074 |
| Journal | European Respiratory Review |
| Volume | 27 |
| Issue number | 150 |
| DOIs | |
| State | Published - Jan 1 2018 |
| Externally published | Yes |
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All Science Journal Classification (ASJC) codes
- Pulmonary and Respiratory Medicine
Cite this
Pharmacological management of progressive-fibrosing interstitial lung diseases : A review of the current evidence. / Richeldi, Luca; Varone, Francesco; Bergna, Miguel; de Andrade, Joao; Falk, Jeremy; Hallowell, Robert; Jouneau, Stéphane; Kondoh, Yasuhiro; Morrow, Lee E.; Randerath, Winfried; Strek, Mary; Tabaj, Gabriela.
In: European Respiratory Review, Vol. 27, No. 150, 180074, 01.01.2018.Research output: Contribution to journal › Review article
}
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 E.
AU - Randerath, Winfried
AU - Strek, Mary
AU - Tabaj, Gabriela
PY - 2018/1/1
Y1 - 2018/1/1
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.
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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
VL - 27
JO - European Respiratory Review
JF - European Respiratory Review
SN - 0905-9180
IS - 150
M1 - 180074
ER -