Universität zu Köln

Richeldi, Luca ORCID: 0000-0001-8594-1448, Varone, Francesco, Bergna, Miguel, de Andrade, Joao, Falk, Jeremy, Hallowell, Robert, Jouneau, Stephane, Kondoh, Yasuhiro, Morrow, Lee, Randerath, Winfried, Strek, Mary and Tabaj, Gabriela (2018). Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence. Eur. Respir. Rev., 27 (150). SHEFFIELD: EUROPEAN RESPIRATORY SOC JOURNALS LTD. ISSN 1600-0617

Full text not available from this repository.

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 WE 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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Richeldi, Luca UNSPECIFIED orcid.org/0000-0001-8594-1448 UNSPECIFIED Varone, Francesco UNSPECIFIED UNSPECIFIED UNSPECIFIED Bergna, Miguel UNSPECIFIED UNSPECIFIED UNSPECIFIED de Andrade, Joao UNSPECIFIED UNSPECIFIED UNSPECIFIED Falk, Jeremy UNSPECIFIED UNSPECIFIED UNSPECIFIED Hallowell, Robert UNSPECIFIED UNSPECIFIED UNSPECIFIED Jouneau, Stephane UNSPECIFIED UNSPECIFIED UNSPECIFIED Kondoh, Yasuhiro UNSPECIFIED UNSPECIFIED UNSPECIFIED Morrow, Lee UNSPECIFIED UNSPECIFIED UNSPECIFIED Randerath, Winfried UNSPECIFIED UNSPECIFIED UNSPECIFIED Strek, Mary UNSPECIFIED UNSPECIFIED UNSPECIFIED Tabaj, Gabriela UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-161622
DOI:	10.1183/16000617.0074-2018
Journal or Publication Title:	Eur. Respir. Rev.
Volume:	27
Number:	150
Date:	2018
Publisher:	EUROPEAN RESPIRATORY SOC JOURNALS LTD
Place of Publication:	SHEFFIELD
ISSN:	1600-0617
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language IDIOPATHIC PULMONARY-FIBROSIS; STEM-CELL TRANSPLANTATION; TYROSINE KINASE INHIBITOR; SYSTEMIC-SCLEROSIS; MYCOPHENOLATE-MOFETIL; RHEUMATOID-ARTHRITIS; PULSE CYCLOPHOSPHAMIDE; OPEN-LABEL; NINTEDANIB; RITUXIMAB Multiple languages Respiratory System Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/16162