Digital ischemia in scleroderma spectrum of diseases

Area of Science:

• Rheumatology and Immunology
• Vascular Biology
• Dermatology

Background:

• Systemic Sclerosis (SSc), or scleroderma, is an autoimmune disease of unknown cause.
• It involves widespread vasculopathy and fibrosis, leading to significant morbidity.
• Digital ulcers (DUs) are a common and serious manifestation of SSc-related vasculopathy.

Purpose of the Study:

• To summarize current knowledge on the pathogenesis of DUs in SSc.
• To review definitions and diagnostic criteria for SSc-related DUs.
• To discuss current management strategies and explore novel therapies for DUs in SSc.

Main Methods:

• Literature review of pathogenesis, definitions, management, and therapies for DUs in SSc.
• Synthesis of information from peer-reviewed studies and clinical guidelines.
• Focus on endothelial dysfunction, intimal proliferation, and thrombosis in SSc vasculopathy.

Main Results:

• DUs are a frequent complication (30% annually) of SSc, linked to endothelial dysfunction.
• Pathogenesis involves intimal proliferation and thrombosis, contributing to digital ischemia.
• Current management focuses on wound care, vasodilators, and anticoagulation.

Conclusions:

• DUs represent a significant clinical challenge in Systemic Sclerosis management.
• Understanding pathogenesis is key to developing targeted and effective therapies.
• Further research into novel treatments is crucial for improving patient outcomes in SSc.