Results 11 to 20 of about 1,628,316 (351)

Pathophysiology of systemic sclerosis (scleroderma)

Kaohsiung Journal of Medical Sciences, 2022
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 endothelial damage, an inflammatory infiltrate and a resulting ...
A. Rosendahl, K. Schönborn, T. Krieg
semanticscholar   +1 more source

Myofibroblast transcriptome indicates SFRP2hi fibroblast progenitors in systemic sclerosis skin

Nature Communications, 2021
Skin and lung fibrosis in systemic sclerosis (SSc) is driven by myofibroblasts, alpha-smooth muscle actin expressing cells. The number of myofibroblasts in SSc skin correlates with the modified Rodnan skin score, the most widely used clinical measure of ...
T. Tabib, Mengqi Huang, N. Morse, A. Papazoglou, Rithika Behera, M. Jia, M. Bulik, Daisy Monier, P. Benos, Wei Chen, R. Domsic, R. Lafyatis   +11 more
semanticscholar   +1 more source

Tocilizumab Prevents Progression of Early Systemic Sclerosis–Associated Interstitial Lung Disease

Arthritis & Rheumatology, 2021
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 terms of radiographically evident quantitative lung involvement.
D. Roofeh, Celia J. F. Lin, J. Goldin, G. Kim, D. Furst, C. Denton, Suiyuan Huang, D. Khanna   +7 more
semanticscholar   +1 more source

The burden of systemic sclerosis in Switzerland – the Swiss systemic sclerosis EUSTAR cohort

Swiss Medical Weekly, 2021
OBJECTIVES Characteristics of Swiss patients with systemic sclerosis have not been described so far. The aim of the current study was to identify unmet needs in comparison with other European countries that could inform specific interventions
Jasmin Hernández, Suzana Jordan, Rucsandra Dobrota, Michele Iudici, Paul Hasler, Camillo Ribi, Peter Villiger, Panayiotis Vlachoyiannopoulos, Alessandra Vacca, Ludmila Garzanova, Alessandro Giollo, Edoardo Rosato, Ina Kötter, Patricia E. Carreira, Andrea Doria, Jörg Henes, Ulf Müller-Ladner, Vanessa Smith, Jörg Distler, Armando Gabrielli, Anna-Maria Hoffman-Vold, Ulrich Walker, Oliver Distler, the EUSTAR collaborators   +23 more
doaj   +1 more source

Current Concepts on the Pathogenesis of Systemic Sclerosis

Clinical reviews in allergy and immunology, 2021
From the clinical standpoint, systemic sclerosis (SSc) is characterized by skin and internal organ fibrosis, diffuse fibroproliferative vascular modifications, and autoimmunity. Clinical presentation and course are highly heterogenous and life expectancy
M. Truchetet, N. Brembilla, C. Chizzolini   +2 more
semanticscholar   +1 more source

Oxidative stress-dependent activation of collagen synthesis is induced in human pulmonary smooth muscle cells by sera from patients with scleroderma-associated pulmonary hypertension [PDF]

, 2014
Pulmonary arterial hypertension is a major complication of systemic sclerosis. Although oxidative stress, intima hyperplasia and a progressive vessel occlusion appear to be clearly involved, the fine molecular mechanisms underpinning the onset and ...
Boin, Francesco, Cossu, Annalisa, Emanueli, Costanza, Erre, Gian Luca, Giordo, Roberta, Passiu, Giuseppe, Pintus, Gianfranco, Posadino, Anna Maria, Spinetti, Gaia   +8 more
core   +8 more sources

French recommendations for the management of systemic sclerosis

Orphanet Journal of Rare Diseases, 2021
Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration.
E. Hachulla, C. Agard, Y. Allanore, J. Avouac, B. Bader-Meunier, A. Belot, A. Bérézné, A. Bouthors, G. Condette-Wojtasik, J. Constans, P. De Groote, E. Diot, F. Dumas, P. Jego, F. Joly, D. Launay, V. le Guern, J. Le Quintrec, G. Lescaille, C. Meune, B. Moulin, C. Nguyen, Nadine Omeish, F. Pène, M. Richard, J. Rochefort, A. Roren, O. Sitbon, V. Sobanski, M. Truchetet, L. Mouthon, Marc Emmanuel Sabine Julia Yoram Benjamin Vincent Bruno Bayen Bergot Berthier Bosco Bouhnik Chaigne Cottin, M. Bayen, E. Bergot, S. Berthier, J. Bosco, Y. Bouhnik, B. Chaigne, V. Cottin, B. Crestani, C. Deligny, V. Descroix, D. Farge, D. Godard, B. Granel, P. Guilpain, B. Imbert, A. le Quellec, C. Lega, C. Lok, H. Maillard, T. Martin, G. Pugnet, V. Queyrel, L. Raffray, F. Rilliard, M. Romier, Lauren Schuller, A. Servettaz   +58 more
semanticscholar   +1 more source

Pathogenesis of Systemic Sclerosis [PDF]

Frontiers in Immunology, 2015
Systemic scleroderma (SSc) is one of the most complex systemic autoimmune diseases. It targets the vasculature, connective tissue-producing cells (namely fibroblasts/myofibroblasts), and components of the innate and adaptive immune systems. Clinical and pathologic manifestations of SSc are the result of: (1) innate/adaptive immune system abnormalities ...
Debendra ePattanaik, Debendra ePattanaik, Monica eBrown, Bradley C. Postlethwaite, Arnold E Postlethwaite, Arnold E Postlethwaite   +5 more
openaire   +4 more sources

Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality

Arthritis Research & Therapy, 2021
Highlights • Male sex, cardiac involvement, DLCO 5 mg/l are strong predictors of mortality in systemic sclerosis. • This study shows the survival of subtypes and in particular sine scleroderma. • Sine scleroderma subtype has better survival than diffuse
Sébastien De Almeida Chaves, Tiphaine Porel, Mickael Mounié, Laurent Alric, Léonardo Astudillo, Antoine Huart, Olivier Lairez, Martin Michaud, Grégoire Prévot, David Ribes, Laurent Sailler, Francis Gaches, Daniel Adoue, Gregory Pugnet   +13 more
doaj   +1 more source

Systemic sclerosis

The Lancet, 2017
Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including ...
Denton, CP, Khanna, D
openaire   +5 more sources