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 …

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 …

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 …

Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

AM Hoffmann-Vold, Y Allanore, M Alves… - Annals of the …, 2021 - ard.bmj.com
Objectives To identify overall disease course, progression patterns and risk factors
predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis …

Nintedanib for systemic sclerosis–associated interstitial lung disease

O Distler, KB Highland, M Gahlemann… - … England Journal of …, 2019 - Mass Medical Soc
Background Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis
and a leading cause of systemic sclerosis–related death. Nintedanib, a tyrosine kinase …

Safety and efficacy of rituximab in systemic sclerosis (DESIRES): a double-blind, investigator-initiated, randomised, placebo-controlled trial

S Ebata, A Yoshizaki, K Oba, K Kashiwabara… - The Lancet …, 2021 - thelancet.com
Background Systemic sclerosis is a connective tissue disease characterised by multiorgan
fibrosis with an autoimmune background and poor prognosis. Although a few drugs have …

Systemic sclerosis-associated interstitial lung disease

A Perelas, RM Silver, AV Arrossi… - The Lancet respiratory …, 2020 - thelancet.com
Systemic sclerosis is an autoimmune connective tissue disease, which is characterised by
immune dysregulation and progressive fibrosis that typically affects the skin, with variable …

Tocilizumab prevents progression of early systemic sclerosis–associated interstitial lung disease

D Roofeh, CJF Lin, J Goldin, GH Kim… - Arthritis & …, 2021 - Wiley Online Library
Objective Tocilizumab (TCZ) has demonstrated lung function preservation in 2 randomized
controlled trials in early systemic sclerosis (SSc). This effect has yet to be characterized in …

Systemic sclerosis–associated interstitial lung disease: How to incorporate two food and drug administration–approved therapies in clinical practice

D Khanna, A Lescoat, D Roofeh… - Arthritis & …, 2022 - Wiley Online Library
Systemic sclerosis (SSc; scleroderma) has the highest individual mortality of all rheumatic
diseases, and interstitial lung disease (ILD) is among the leading causes of SSc‐related …

Progressive pulmonary fibrosis: an expert group consensus statement

SK Rajan, V Cottin, R Dhar, S Danoff… - European …, 2023 - Eur Respiratory Soc
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 …