Related Experiment Video

Updated: Apr 24, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

22.1K

Pathophysiology of systemic sclerosis: state of the art in 2014

Nicolas Dumoitier1, Sébastien Lofek2, Luc Mouthon3

  • 1Institut Cochin, Inserm U1016, CNRS UMR 8104, 8, rue Méchain, 75014 Paris, France; Université Paris Diderot, 75013 Paris, France.

Presse Medicale (Paris, France : 1983)
|September 3, 2014
PubMed

Insights

Systemic sclerosis (SSc) pathogenesis is better understood through new models and identified hallmarks like reactive oxygen species and interferon signatures. Research highlights the roles of B cells, autoantibodies, and plasmacytoid dendritic cells in SSc development.

Area of Science:

  • Immunology
  • Pathogenesis of Systemic Sclerosis

Background:

  • Systemic sclerosis (SSc) pathogenesis requires further elucidation.
  • New experimental models are crucial for understanding disease mechanisms and therapeutic targets.

Purpose of the Study:

  • To review recent advancements in understanding SSc pathogenesis.
  • To highlight key cellular and molecular players involved in SSc development.

Main Methods:

  • Review of experimental models for SSc.
  • Analysis of genetic studies and molecular pathways.
  • Investigation of cellular roles including fibroblasts, endothelial cells, B cells, and dendritic cells.

Main Results:

  • Reactive oxygen species are identified as a hallmark in SSc fibroblasts and endothelial cells.
  • An interferon signature has been identified as a significant finding in SSc genetics.
  • B cells, autoantibodies, and plasmacytoid dendritic cells (secreting CXCL4) are implicated in SSc pathogenesis.

Conclusions:

  • Significant progress has been made in understanding SSc pathogenesis.
  • Further research is needed to confirm the role of plasmacytoid dendritic cells and CXCL4 in SSc.

More Related Videos

Vasodilation of Isolated Vessels and the Isolation of the Extracellular Matrix of Tight-skin Mice08:09

Vasodilation of Isolated Vessels and the Isolation of the Extracellular Matrix of Tight-skin Mice

Published on: March 24, 2017

7.3K
Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma10:21

Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma

Published on: September 20, 2024

913

Related Concept Videos

Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
40
The JAK-STAT Signaling Pathway01:20

The JAK-STAT Signaling Pathway

Several cytokine receptors have tightly bound Janus kinase or JAK proteins attached at their cytosolic tail. Small signaling molecules such as cytokines, growth hormones, or prolactins bind to the cytokine receptors and initiate their dimerization. The dimerization brings the cytosolic JAKs together that trans-phosphorylate and activates each other. The activated JAKs now phosphorylate cytosolic tails of the cytokine receptors, which serve as binding sites for adaptor proteins such as  SH2...
10.1K
Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
897
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
932
Atherosclerosis I: Introduction01:30

Atherosclerosis I: Introduction

Atherosclerosis is a progressive disorder characterized by the buildup of plaques on the arterial inner wall, causing them to narrow and harden over time. These plaques comprise lipids, calcium, blood components, carbohydrates, and fibrous tissue. The process primarily affects the intima of large and medium-sized arteries, reducing blood flow in any artery.Etiology and risk factorsThe cause of atherosclerosis is multifactorial, involving a complex interplay among endothelial injury, lipid...
2.3K
Pathophysiology of Heart Failure01:17

Pathophysiology of Heart Failure

Heart failure (HF) is a progressive syndrome involving ventricles that leads to inadequate cardiac output. It can be classified based on location and output or ejection fraction. Ejection fraction (EF) is an essential measurement in the diagnosis and surveillance of HF. Reduced EF corresponds to systolic heart failure (HFrEF). However, HF with preserved ejection fraction (HFpEF) is becoming increasingly prevalent. Also known as diastolic HF, this form of HF is related to aging. The...
4.6K