[Pro-angiogenic cytokines in systemic sclerosis].

Area of Science:

• Connective tissue diseases
• Fibrosis and microvasculopathy
• Angiogenesis and vasculogenesis

Background:

• Systemic sclerosis (SSc) is a complex disease marked by progressive fibrosis.
• SSc features microvasculopathy, indicating issues with blood vessel formation and repair.
• Despite elevated levels of certain growth factors, the body's ability to form new blood vessels is compromised in SSc.

Purpose of the Study:

• To review the role of key proangiogenic factors in Systemic Sclerosis.
• To explore the paradoxical impairment of angiogenesis and vasculogenesis in SSc.
• To discuss the implications of these angiogenic dysregulations in SSc pathophysiology.

Main Methods:

• Literature review of studies on proangiogenic factors in Systemic Sclerosis.
• Analysis of the expression and function of vascular endothelial growth factor (VEGF), placental growth factor (PlGF), endoglin, platelet-derived growth factor (PDGF), endothelin-1, angiopoietins, stromal cell-derived factor-1 (SDF-1), and urokinase plasminogen activator receptor (uPAR).
• Discussion of the balance between proangiogenic signaling and the resulting vascular response in SSc.

Main Results:

• Elevated levels of multiple proangiogenic factors, including VEGF, PlGF, endoglin, PDGF, endothelin-1, angiopoietins, SDF-1, and uPAR, are observed in SSc.
• Despite the abundance of these factors, compensatory angiogenesis and vasculogenesis are significantly impaired in SSc.
• This leads to a paradoxical inadequate angiogenic response, contributing to the disease's microvasculopathy.

Conclusions:

• Systemic sclerosis exhibits a complex dysregulation of angiogenic pathways.
• The impaired vascular repair in SSc is not due to a lack of proangiogenic signals but rather an inadequate response to them.
• Understanding these angiogenic paradoxes is crucial for developing targeted therapies for SSc.