About this Research Topic
Systemic Sclerosis (SSc) is the most severe connective tissue disease that currently has no cure and a high morbimortality. SSc is characterized by a high degree of fibrosis, affecting the skin and various internal organs (i.e. lung and heart fibrosis), in a context of diffuse vasculopathy (Raynaud’s phenomenon and pulmonary hypertension) and abnormal immune system activation, in which both innate and adaptive immunity is activated. Beyond this activation, the immune system is also thought to play a role in the pathophysiology of SSc, which is characterized by a complex interplay between the immune system, vascular abnormalities and fibroblasts activation. Activated immune cells can interact in different ways with targeted cells like fibroblasts and endothelial cells in SSc: direct cell-cell contact, production of profibrotic and proinflammatory cytokines, growth factors and autoantibodies. Yet, this field needs careful assessment, as there are many gaps in our knowledge, which is the topic of this collection.
The objective of the collection is to address the issue of the role of the immune system in the pathophysiology of SSc. There is already significant data regarding the fibrotic and vascular part of the disease. Yet, the description and understanding on how immune cells are altered and interact with targeted cells like fibroblasts and endothelial cells is more limited. Describing the modifications of circulating and tissue immune cells and their role in the pathophysiology of SSc is very important to understand if, when and how the immune cells could be therapeutic targets.
The scope is to focus on the pathophysiological role of immune cells in SSc. We welcome Original Research and Review articles focusing on, but not limited to, the following areas:
• Role of innate immunity in the pathophysiology of SSc (human and experimental models)
• Role of cellular immunity in the pathophysiology of SSc (human and experimental models)Role of humoral immunity in the pathophysiology of SSc (human and experimental models)
Keywords: systemic sclerosis, fibrosis, pathophysiology, immune cells, fibroblasts, endothelial cells
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