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Macro- and microvascular disease in systemic sclerosis
Niloufar Kavian1, Frédéric Batteux1
1Faculté de Médecine Paris Descartes, Sorbonne Paris Cité, INSERM U 1016, Institut Cochin, Paris, France; Laboratoire d'immunologie biologique, Hôpital Cochin, Groupe Hospitalier Paris Centre, AP-HP, 75679 Paris cedex 14, France.
Insights
Vasculopathy is a common issue in connective tissue diseases, particularly systemic sclerosis (SSc). This review details SSc-related vascular problems and their underlying disease mechanisms.
Area of Science:
- Rheumatology
- Vascular Medicine
- Immunology
Background:
- Connective tissue diseases frequently involve vasculopathy, impacting disease pathogenesis.
- Systemic sclerosis (SSc) is an autoimmune disorder causing organ fibrosis and significant vascular disease.
- Both micro- and macro-vessels are primary targets in SSc.
Purpose of the Study:
- To review the clinical manifestations of vasculopathy in SSc patients.
- To discuss the pathophysiology of vascular disorders in systemic sclerosis.
Main Methods:
- Literature review of clinical manifestations.
- Review of pathophysiological mechanisms of vascular damage.
Main Results:
- Vasculopathy presents diverse clinical symptoms in SSc patients.
- Pathophysiology involves direct targeting of blood vessels in SSc.
- Fibrosis and autoimmune processes contribute to vascular complications.
Conclusions:
- Vasculopathy is a key feature of systemic sclerosis, affecting various vessel types.
- Understanding SSc-related vasculopathy is crucial for patient management.
- Further research into pathophysiology can guide therapeutic strategies.
Abstract:
Vasculopathy is common in patients with connective tissue disease and can be directly implicated in the pathogenesis of the disease. Systemic sclerosis is an auto-immune multiorgan connective tissue disorder characterized by fibrosis of the skin and visceral organs and vascular disease. Micro- and macro-vessels are a direct target of the disease. In this review, we present the various clinical manifestations of the vasculopathy that can be present in SSc patients, and then discuss the various aspects of the pathophysiology of the vascular disorders.
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