Pathogenesis of systemic sclerosis

D Pattanaik, M Brown, BC Postlethwaite… - Frontiers in …, 2015‏ - frontiersin.org
Systemic scleroderma (SSc) is one of the most complex systemic autoimmune diseases. It
targets the vasculature, connective tissue-producing cells (namely fibroblasts/myofibroblasts …

IRF7: role and regulation in immunity and autoimmunity

W Ma, G Huang, Z Wang, L Wang, Q Gao - Frontiers in immunology, 2023‏ - frontiersin.org
Interferon regulatory factor (IRF) 7 was originally identified as master transcriptional factor
that produced IFN-I and regulated innate immune response, subsequent studies have …

Myofibroblast transcriptome indicates SFRP2hi fibroblast progenitors in systemic sclerosis skin

T Tabib, M Huang, N Morse, A Papazoglou… - Nature …, 2021‏ - nature.com
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 …

Type 1 interferon activation in systemic sclerosis: a biomarker, a target or the culprit

V Kakkar, S Assassi, Y Allanore… - Current opinion in …, 2022‏ - journals.lww.com
The role played by T1 IFN and its regulatory genes in SSc is becoming increasingly evident
and strikingly similar to the role observed in SLE. This observation, together with the benefit …

[HTML][HTML] Type I interferons in autoimmunity

R Fernandez-Ruiz, TB Niewold - Journal of Investigative Dermatology, 2022‏ - Elsevier
Dysregulated IFN-1 responses play crucial roles in the development of multiple forms of
autoimmunity. Many patients with lupus, systemic sclerosis, Sjogren's syndrome, and …

[HTML][HTML] Type I interferons in autoimmunity: implications in clinical phenotypes and treatment response

AC Londe, R Fernandez-Ruiz, PR Julio… - The Journal of …, 2023‏ - jrheum.org
Type I interferon (IFN-I) is thought to play a role in many systemic autoimmune diseases. IFN-
I pathway activation is associated with pathogenic features, including the presence of …

Systems level analysis of systemic sclerosis shows a network of immune and profibrotic pathways connected with genetic polymorphisms

JM Mahoney, J Taroni, V Martyanov… - PLoS computational …, 2015‏ - journals.plos.org
Systemic sclerosis (SSc) is a rare systemic autoimmune disease characterized by skin and
organ fibrosis. The pathogenesis of SSc and its progression are poorly understood. The SSc …

Interferon regulatory factor 7 (IRF7) represents a link between inflammation and fibrosis in the pathogenesis of systemic sclerosis

M Wu, B Skaug, X Bi, T Mills, G Salazar… - Annals of the …, 2019‏ - ard.bmj.com
Objectives There is considerable evidence that implicates dysregulation of type I interferon
signalling (or type I IFN signature) in the pathogenesis of systemic sclerosis (SSc). Interferon …

New insights into the genetics and epigenetics of systemic sclerosis

C Angiolilli, W Marut, M van der Kroef… - Nature Reviews …, 2018‏ - nature.com
Systemic sclerosis (SSc) is a severe autoimmune disease that is characterized by vascular
abnormalities, immunological alterations and fibrosis of the skin and internal organs. The …

Unfolding the pathogenesis of scleroderma through genomics and epigenomics

PS Tsou, AH Sawalha - Journal of autoimmunity, 2017‏ - Elsevier
With unknown etiology, scleroderma (SSc) is a multifaceted disease characterized by
immune activation, vascular complications, and excessive fibrosis in internal organs …