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 …

Interstitial lung disease: a review

TM Maher - JAMA, 2024 - jamanetwork.com
Importance Interstitial lung disease (ILD) consists of a group of pulmonary disorders
characterized by inflammation and/or fibrosis of the lung parenchyma associated with …

Progressive pulmonary fibrosis: an expert group consensus statement

SK Rajan, V Cottin, R Dhar, S Danoff… - European …, 2023 - publications.ersnet.org
This expert group consensus statement emphasises the need for standardising the definition
of progressive fibrosing interstitial lung diseases (F-ILDs), with an accurate initial diagnosis …

State-of-the-art evidence in the treatment of systemic sclerosis

JE Pope, CP Denton, SR Johnson… - Nature Reviews …, 2023 - nature.com
Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multi-organ
involvement, fibrosis and vasculopathy. Treatment in SSc, including early diffuse cutaneous …

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 …

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 …

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 …

Expert consensus on the management of systemic sclerosis-associated interstitial lung disease

FF Rahaghi, VM Hsu, RJ Kaner, MD Mayes… - Respiratory …, 2023 - Springer
Abstract Background Systemic sclerosis (SSc) is a rare, complex, connective tissue disorder.
Interstitial lung disease (ILD) is common in SSc, occurring in 35–52% of patients and …

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 …