Unraveling SSc Pathophysiology; The Myofibroblast

Area of Science:

• Immunology
• Rheumatology
• Cell Biology

Background:

• Systemic sclerosis (SSc) is a severe autoimmune disease with high morbidity and mortality.
• Current treatments for SSc lack disease-modifying capabilities.
• Myofibroblasts are central to SSc pathophysiology, producing excessive extracellular matrix like collagen type I.

Purpose of the Study:

• To review the presence, formation, and function of myofibroblasts in Systemic sclerosis.
• To elucidate the role of innate immune cells, such as mast cells and T helper 2 lymphocytes, in mediating myofibroblast activity in SSc.
• To discuss emerging therapeutic strategies targeting myofibroblasts for SSc.

Main Methods:

• This is a narrative review, synthesizing existing literature.
• The review focuses on the cellular and molecular mechanisms underlying myofibroblast involvement in SSc.
• It examines the interplay between immune cells and myofibroblasts.

Main Results:

• Myofibroblasts are key effector cells in SSc, contributing to tissue fibrosis.
• Innate immune cells, including mast cells and T helper 2 lymphocytes, significantly stimulate and mediate myofibroblast activation and function in SSc.
• Understanding these interactions provides a basis for novel therapeutic interventions.

Conclusions:

• Myofibroblasts are critical drivers of fibrosis in Systemic sclerosis.
• Immune system components play a crucial role in regulating myofibroblast behavior in SSc.
• Targeting myofibroblasts represents a promising future therapeutic avenue for SSc management.