Pathophysiology of systemic sclerosis: current understanding and new insights

M Cutolo, S Soldano, V Smith - Expert review of clinical …, 2019 - Taylor & Francis
ABSTRACT Introduction: Systemic sclerosis (SSc) is a complex autoimmune connective
tissue disease characterized by chronic and progressive tissue and organ fibrosis with …

Integrating mechanisms of pulmonary fibrosis

TA Wynn - Journal of Experimental Medicine, 2011 - rupress.org
Pulmonary fibrosis is a highly heterogeneous and lethal pathological process with limited
therapeutic options. Although research on the pathogenesis of pulmonary fibrosis has …

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 …

Single-cell analysis reveals fibroblast heterogeneity and myofibroblasts in systemic sclerosis-associated interstitial lung disease

E Valenzi, M Bulik, T Tabib, C Morse… - Annals of the …, 2019 - ard.bmj.com
Objectives Myofibroblasts are key effector cells in the extracellular matrix remodelling of
systemic sclerosis-associated interstitial lung disease (SSc-ILD); however, the diversity of …

A circulating cell population showing both M1 and M2 monocyte/macrophage surface markers characterizes systemic sclerosis patients with lung involvement

AC Trombetta, S Soldano, P Contini, V Tomatis… - Respiratory …, 2018 - Springer
Abstract Background Systemic sclerosis (SSc) is a disorder characterized by immune system
alterations, vasculopathy and fibrosis. SSc-related interstitial lung disease (ILD) represents a …

The immunopathogenesis of fibrosis in systemic sclerosis

M Brown, S O'Reilly - Clinical & Experimental Immunology, 2019 - academic.oup.com
Systemic sclerosis (SSc) is an idiopathic systemic autoimmune disease. It is characterized
by a triad of hallmarks: immune dysfunction, fibrosis and vasculopathy. Immune dysfunction …

[HTML][HTML] Cytokine mediated tissue fibrosis

LA Borthwick, TA Wynn, AJ Fisher - … acta (BBA)-molecular basis of disease, 2013 - Elsevier
Acute inflammation is a recognised part of normal wound healing. However, when
inflammation fails to resolve and a chronic inflammatory response is established this …

Cellular interactions in the pathogenesis of interstitial lung diseases

G Bagnato, S Harari - European Respiratory Review, 2015 - Eur Respiratory Soc
Interstitial lung disease (ILD) encompasses a large and diverse group of pathological
conditions that share similar clinical, radiological and pathological manifestations, despite …

Genetics and genomics of pulmonary arterial hypertension

F Soubrier, WK Chung, R Machado, E Grünig… - Journal of the American …, 2013 - jacc.org
Major discoveries have been obtained within the last decade in the field of hereditary
predisposition to pulmonary arterial hypertension (PAH). Among them, the identification of …

The Th17 pathway and inflammatory diseases of the intestines, lungs, and skin

CT Weaver, CO Elson, LA Fouser… - Annual Review of …, 2013 - annualreviews.org
The recent discovery of a new CD4+ T cell subset, Th17, has transformed our understanding
of the pathogenetic basis of an increasing number of chronic immune-mediated diseases …