Vascular complications of scleroderma

Area of Science:

• Vascular Biology
• Rheumatology
• Dermatology

Background:

• Systemic sclerosis (SSc) is a multiorgan autoimmune disease characterized by vascular wall injury and microvascular damage.
• This damage manifests as megacapillaries, avascular areas, and reduced capillary density, leading to clinical issues like painful digital ulcers.
• Despite impaired blood flow and low oxygen levels, effective angiogenesis is notably absent in SSc skin.

Purpose of the Study:

• To investigate the disturbed angiogenesis and progressive vascular damage in Systemic Sclerosis.
• To highlight the link between microvascular changes and clinical manifestations such as digital ulcers and organ complications.

Main Methods:

• Analysis of microvascular changes in SSc patients using Nailfold Video-Capillaroscopy.
• Observation of progressive vascular damage evolution from early to late stages.
• Correlation of vascular pathology with clinical manifestations including Raynaud's phenomenon and digital ischemia.

Main Results:

• Nailfold Video-Capillaroscopy reveals distinct morphological changes in SSc vasculature, indicating disturbed angiogenesis.
• Progressive vascular damage is observed, evolving through different stages.
• Raynaud's phenomenon and structural vasculopathy contribute significantly to digital ulceration and critical ischemia.

Conclusions:

• Angiogenesis is profoundly disturbed in Systemic Sclerosis, contributing to significant morbidity.
• Vascular damage in SSc affects multiple organ systems, leading to severe complications like pulmonary arterial hypertension and renal crisis.
• The vascular pathology in SSc underlies its high mortality rate due to organ-based complications.