Spectrum of fibrotic lung diseases

M Wijsenbeek, V Cottin - New England Journal of Medicine, 2020 - Mass Medical Soc
Pulmonary Fibrotic Diseases This review covers fibrotic pulmonary diseases. Although
idiopathic pulmonary fibrosis is very common, the review focuses mainly on fibrotic diseases …

Fibrosing interstitial lung diseases: knowns and unknowns

V Cottin, L Wollin, A Fischer… - European …, 2019 - Eur Respiratory Soc
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 …

Pirfenidone: molecular mechanisms and potential clinical applications in lung disease

SM Ruwanpura, BJ Thomas… - American journal of …, 2020 - atsjournals.org
Pirfenidone (PFD) is a pharmacological compound with therapeutic efficacy in idiopathic
pulmonary fibrosis. It has been chiefly characterized as an antifibrotic agent, although it was …

[HTML][HTML] The natural history of progressive fibrosing interstitial lung diseases

M Kolb, M Vašáková - Respiratory research, 2019 - Springer
A proportion of patients with certain types of interstitial lung disease (ILD), including chronic
hypersensitivity pneumonitis and ILDs associated with autoimmune diseases, develop a …

Inhaled treprostinil and forced vital capacity in patients with interstitial lung disease and associated pulmonary hypertension: a post-hoc analysis of the INCREASE …

SD Nathan, A Waxman, S Rajagopal… - The Lancet …, 2021 - thelancet.com
Background INCREASE was a randomised, placebo-controlled, phase 3 trial that evaluated
inhaled treprostinil in patients with interstitial lung disease (ILD) and associated pulmonary …

Pirfenidone reduces respiratory-related hospitalizations in idiopathic pulmonary fibrosis

B Ley, J Swigris, B Day, JL Stauffer… - American journal of …, 2017 - atsjournals.org
Rationale: Respiratory-related hospitalizations of patients with idiopathic pulmonary fibrosis
(IPF) are more frequent than those for acute IPF exacerbations and are associated with poor …

[HTML][HTML] Nintedanib in children and adolescents with fibrosing interstitial lung diseases

R Deterding, LR Young, EM DeBoer… - European …, 2023 - Eur Respiratory Soc
Background Childhood interstitial lung disease (ILD) comprises a spectrum of rare ILDs
affecting infants, children and adolescents. Nintedanib is a licensed treatment for pulmonary …

Effect of antimicrobial therapy on respiratory hospitalization or death in adults with idiopathic pulmonary fibrosis: the CleanUP-IPF randomized clinical trial

FJ Martinez, E Yow, KR Flaherty, LD Snyder… - Jama, 2021 - jamanetwork.com
Importance Alteration in lung microbes is associated with disease progression in idiopathic
pulmonary fibrosis. Objective To assess the effect of antimicrobial therapy on clinical …

First-in-human high-cumulative-dose stem cell therapy in idiopathic pulmonary fibrosis with rapid lung function decline

A Averyanov, I Koroleva… - Stem cells …, 2020 - academic.oup.com
Previous phase I studies demonstrated safety and some beneficial effects of mesenchymal
stem cells (MSCs) in patients with mild to moderate idiopathic pulmonary fibrosis (IPF). The …

Immunomodulatory treatment of interstitial lung disease

L van den Bosch, F Luppi, G Ferrara… - … in Respiratory Disease, 2022 - journals.sagepub.com
Interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) have an array
of immunomodulatory treatment options compared with IPF, due to their inflammatory …