Systemic sclerosis

ER Volkmann, K Andréasson, V Smith - The Lancet, 2023 - thelancet.com
Systemic sclerosis, also known as scleroderma, is a rare and complex autoimmune
connective-tissue disease. Once considered an untreatable and unpredictable condition …

Interstitial lung diseases

M Wijsenbeek, A Suzuki, TM Maher - The Lancet, 2022 - thelancet.com
Over 200 interstitial lung diseases, from ultra rare to relatively common, are recognised.
Most interstitial lung diseases are characterised by inflammation or fibrosis within the …

2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with …

SR Johnson, EJ Bernstein, MB Bolster… - Arthritis & …, 2024 - Wiley Online Library
Objective We provide evidence‐based recommendations regarding the treatment of
interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases …

Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo …

J Behr, A Prasse, M Kreuter, J Johow… - The Lancet …, 2021 - thelancet.com
Background Pirfenidone has been shown to slow disease progression in patients with
idiopathic pulmonary fibrosis (IPF). However, there are few treatment options for progressive …

Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial

D Khanna, CJF Lin, DE Furst, J Goldin… - The Lancet …, 2020 - thelancet.com
Background A phase 2 trial of tocilizumab showed preliminary evidence of efficacy in
systemic sclerosis. We assessed skin fibrosis and systemic sclerosis-associated interstitial …

Interstitial lung abnormalities detected incidentally on CT: a Position Paper from the Fleischner Society

H Hatabu, GM Hunninghake, L Richeldi… - The lancet Respiratory …, 2020 - thelancet.com
The term interstitial lung abnormalities refers to specific CT findings that are potentially
compatible with interstitial lung disease in patients without clinical suspicion of the disease …

The role of myofibroblasts in physiological and pathological tissue repair

R Schuster, F Younesi, M Ezzo… - Cold Spring Harbor …, 2023 - cshperspectives.cshlp.org
Myofibroblasts are the construction workers of wound healing and repair damaged tissues
by producing and organizing collagen/extracellular matrix (ECM) into scar tissue. Scar tissue …

Nintedanib in patients with progressive fibrosing interstitial lung diseases—subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised …

AU Wells, KR Flaherty, KK Brown, Y Inoue… - The lancet Respiratory …, 2020 - thelancet.com
Background The INBUILD trial investigated the efficacy and safety of nintedanib versus
placebo in patients with progressive fibrosing interstitial lung diseases (ILDs) other than …

Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial

TM Maher, TJ Corte, A Fischer, M Kreuter… - The lancet Respiratory …, 2020 - thelancet.com
Background At present, no approved pharmacotherapies are available for unclassifiable
interstitial lung disease (ILD), which is characterised by progressive fibrosis of the lung. We …

Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities

PM George, P Spagnolo, M Kreuter… - The lancet Respiratory …, 2020 - thelancet.com
Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who
have inexorable progression of pulmonary fibrosis despite treatment, which is known as the …