New insights into the genetics and epigenetics of systemic sclerosis

C Angiolilli, W Marut, M van der Kroef… - Nature Reviews …, 2018 - nature.com
Systemic sclerosis (SSc) is a severe autoimmune disease that is characterized by vascular
abnormalities, immunological alterations and fibrosis of the skin and internal organs. The …

Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised …

D Visca, L Mori, V Tsipouri, S Fleming… - The Lancet …, 2018 - thelancet.com
Background In fibrotic interstitial lung diseases, exertional breathlessness is strongly linked
to health-related quality of life (HRQOL). Breathlessness is often associated with oxygen …

[HTML][HTML] Pirfenidone and nintedanib modulate properties of fibroblasts and myofibroblasts in idiopathic pulmonary fibrosis

ST Lehtonen, A Veijola, H Karvonen… - Respiratory …, 2016 - Springer
Background Idiopathic pulmonary fibrosis (IPF) is an incurable lung disease with a poor
prognosis. Fibroblasts and myofibroblasts are the key cells in the fibrotic process. Recently …

[HTML][HTML] Inhibition of lung microbiota-derived proapoptotic peptides ameliorates acute exacerbation of pulmonary fibrosis

CN D'Alessandro-Gabazza, T Yasuma… - Nature …, 2022 - nature.com
Idiopathic pulmonary fibrosis is an incurable disease of unknown etiology. Acute
exacerbation of idiopathic pulmonary fibrosis is associated with high mortality. Excessive …

[HTML][HTML] Baseline characteristics and survival of patients of idiopathic pulmonary fibrosis: a longitudinal analysis of the Swedish IPF Registry

J Gao, D Kalafatis, L Carlson, IHA Pesonen, CX Li… - Respiratory …, 2021 - Springer
Background Observational data under real-life conditions in idiopathic pulmonary fibrosis
(IPF) is scarce. We explored anti-fibrotic treatment, disease severity and phenotypes in …

[HTML][HTML] A Staphylococcus pro-apoptotic peptide induces acute exacerbation of pulmonary fibrosis

CN D'Alessandro-Gabazza, T Kobayashi… - Nature …, 2020 - nature.com
Idiopathic pulmonary fibrosis (IPF) is a chronic and fatal disease of unknown etiology;
however, apoptosis of lung alveolar epithelial cells plays a role in disease progression. This …

Patients with fibrotic interstitial lung disease hospitalized for acute respiratory worsening: a large cohort analysis

T Moua, BD Westerly, MM Dulohery, CE Daniels… - Chest, 2016 - Elsevier
Background Acute respiratory worsening (ARW) requiring hospitalization in patients with
fibrotic interstitial lung disease (f-ILD) is common. Little is known about the frequency and …

Phenotypic drug screening in a human fibrosis model identified a novel class of antifibrotic therapeutics

M Gerckens, K Schorpp, F Pelizza, M Wögrath… - Science …, 2021 - science.org
Fibrogenic processes instigate fatal chronic diseases leading to organ failure and death.
Underlying biological processes involve induced massive deposition of extracellular matrix …

[HTML][HTML] A homozygous SFTPA1 mutation drives necroptosis of type II alveolar epithelial cells in patients with idiopathic pulmonary fibrosis

A Takezaki, S Tsukumo, Y Setoguchi… - Journal of Experimental …, 2019 - rupress.org
Idiopathic pulmonary fibrosis (IPF) is a fatal disease characterized by scattered fibrotic
lesions in the lungs. The pathogenesis and genetic basis of IPF remain poorly understood …

New frontiers in fibrotic disease therapies: The focus of the Joan and Joel Rosenbloom Center for Fibrotic Diseases at Thomas Jefferson University

J Rosenbloom, S Ren, E Macarak - Matrix Biology, 2016 - Elsevier
Fibrotic diseases constitute a world-wide major health problem, but research support
remains inadequate in comparison to the need. Although considerable understanding of the …