The Role of the Immune System in Systemic Sclerosis

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Immunology and Immunotherapy".

Deadline for manuscript submissions: closed (30 June 2023) | Viewed by 2096

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Special Issue Information

Dear Colleagues,

Systemic sclerosis is a systemic inflammatory disease that begins with immune abnormalities and leads to vascular damage and fibrosis. Although basic research has typically focused on B cells, vascular endothelial cells, and fibroblasts, systemic sclerosis research has made rapid progress, especially in recent years. Reasons for these advances include technological advances such as single cell analysis, as well as advances in clinical and translational research. These rapid advances have left no doubt surrounding the involvement of inflammatory cytokines and immune abnormalities in the pathogenesis of systemic sclerosis. This Special Issue, entitled "The Role of the Immune System in Systemic Sclerosis", aims to advance research on the involvement of the immune system in systemic sclerosis research.

Dr. Takemichi Fukasawa
Guest Editor

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Keywords

  • systemic sclerosis
  • interstitial lung disease
  • pulmonary hypertension
  • ulcer
  • comorbidities
  • B cells
  • T cells
  • macrophages
  • translational research
  • basic research

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Published Papers (1 paper)

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Research

16 pages, 3509 KiB  
Article
Profibrotic Effects of Endothelin-1 on Fibroblasts Are Mediated by Aldosterone In Vitro: Relevance to the Pathogenesis and Therapy of Systemic Sclerosis and Pulmonary Arterial Hypertension
by Giuseppe Argentino, Alessandro Barbieri, Ruggero Beri, Caterina Bason, Andrea Ruzzenente, Oliviero Olivieri, Elisa Tinazzi, Antonio Puccetti, Claudio Vitali, Nicoletta Del Papa, Simonetta Friso and Claudio Lunardi
Biomedicines 2022, 10(11), 2765; https://doi.org/10.3390/biomedicines10112765 - 31 Oct 2022
Cited by 4 | Viewed by 1740
Abstract
Endothelin-1 (ET-1) is a vasoactive and profibrotic peptide that plays a pivotal role in diseases such as systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH), by inducing fibrosis and vascular remodeling. Such effects may be sustained by the induction of aldosterone production and [...] Read more.
Endothelin-1 (ET-1) is a vasoactive and profibrotic peptide that plays a pivotal role in diseases such as systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH), by inducing fibrosis and vascular remodeling. Such effects may be sustained by the induction of aldosterone production and reactive oxygen species (ROS). We have used fibroblasts obtained from skin of healthy donors and SSc patients and commercial fibroblasts from lung to evaluate whether ET-1 is able to stimulate ROS production directly or indirectly through aldosterone induction. We found that ET-1 receptors are present in all types of fibroblasts analyzed, whereas the expression of mineralocorticoid receptor (MCR) is lower in dermal fibroblasts from healthy donors (HDFs) compared to fibroblasts derived from lung (HPFs) or from skin of SSc patients (SScHDFs). ET-1 induces ROS production in HDFs and SScHDFs after 24 h of incubation involving its receptor B (ETB), whereas aldosterone exerts its effects after 40 min of incubation. Moreover, ROS production was inhibited by the pre-incubation of cells with MCR inhibitor. Our results indicate that ET-1 induces ROS indirectly through aldosterone production suggesting that aldosterone may play a pivotal role in the pathogenesis of SSc and PAH. Full article
(This article belongs to the Special Issue The Role of the Immune System in Systemic Sclerosis)
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