[Large vessels vasculopathy in systemic sclerosis]

Area of Science:

• Rheumatology
• Vascular Medicine
• Internal Medicine

Context:

• Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis, vascular dysfunction, and immune dysregulation.
• Vasculopathy in SSc classically involves microcirculation but can also affect proximal large vessels in the limbs.
• Differential diagnosis of large vessel involvement in SSc includes vasculitis and atherosclerosis, posing diagnostic challenges.

Purpose:

• To review the understudied manifestation of large vessel involvement in systemic sclerosis.
• To highlight the prognostic and therapeutic implications of large vessel vasculopathy in SSc.
• To emphasize the importance of early and accurate diagnosis for timely treatment initiation.

Summary:

• Systemic sclerosis can lead to severe vasculopathy affecting not only microcirculation but also proximal large vessels.
• This large vessel involvement in SSc, distinct from microvascular disease, can be challenging to differentiate from other conditions like vasculitis or atherosclerosis.
• Prompt diagnosis and appropriate treatment are essential for managing this severe, potentially irreversible complication and preventing outcomes like amputation.

Impact:

• Improved understanding of large vessel vasculopathy in SSc can lead to earlier diagnosis and intervention.
• Timely management of SSc-related large vessel disease may prevent severe complications such as limb loss.
• This review underscores the need for further research into the mechanisms and treatment strategies for large vessel involvement in systemic sclerosis.