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 …

Shared and distinct mechanisms of fibrosis

JHW Distler, AH Györfi, M Ramanujam… - Nature Reviews …, 2019 - nature.com
Fibrosis is defined as an excessive deposition of connective tissue components and can
affect virtually every organ system, including the skin, lungs, liver and kidney. Fibrotic 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 …

[HTML][HTML] 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 …

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 …

Pathophysiology of systemic sclerosis (scleroderma)

AH Rosendahl, K Schönborn… - The Kaohsiung journal of …, 2022 - Wiley Online Library
Systemic sclerosis (scleroderma) is an autoimmune‐triggered chronic fibrosing disease that
affects the skin and many other organs. Its pathophysiology is complex and involves an early …

[HTML][HTML] Current concepts on the pathogenesis of systemic sclerosis

ME Truchetet, NC Brembilla, C Chizzolini - Clinical reviews in allergy & …, 2023 - Springer
From the clinical standpoint, systemic sclerosis (SSc) is characterized by skin and internal
organ fibrosis, diffuse fibroproliferative vascular modifications, and autoimmunity. Clinical …

The promise of precision medicine in rheumatology

JM Guthridge, CA Wagner, JA James - Nature medicine, 2022 - nature.com
Systemic autoimmune rheumatic diseases (SARDs) exhibit extensive heterogeneity in
clinical presentation, disease course, and treatment response. Therefore, precision …

Combined anti-fibrotic and anti-inflammatory properties of JAK-inhibitors on macrophages in vitro and in vivo: perspectives for scleroderma-associated interstitial lung …

A Lescoat, M Lelong, M Jeljeli, C Piquet-Pellorce… - Biochemical …, 2020 - Elsevier
Janus kinase (JAK) inhibitors (also termed Jakinibs) constitute a family of small drugs that
target various isoforms of JAKs (JAK1, JAK2, JAK3 and/or tyrosine kinase 2 (Tyk2)). They …