A journey to vasculopathy in systemic sclerosis: focus on haemostasis and thrombosis.
Clin Exp Med
; 23(8): 4057-4064, 2023 Dec.
Article
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| MEDLINE
| ID: mdl-37914967
ABSTRACT
Systemic sclerosis is a multisystem connective tissue disease, characterized by endothelial autoimmune activation, along with tissue and vascular fibrosis leading to vasculopathy and to a progressive loss of angiogenesis. This condition further deranges the endothelial barrier favouring the opening of the endothelial junctions allowing the vascular leak in the surrounding tissues this process may induce cell detachment which allows the contact between platelets and collagen present in the exposed subendothelial layer. Platelets first adhere to collagen via glycoprotein VI and then, immediately aggregate because of the release of von Willebrand factor which is a strong activator of platelet aggregation. Activated platelets exert their procoagulant activity, exposing on their membrane phospholipids and phosphatidylserine, enabling the adsorption of clotting factors ready to form thrombin which in turn drives the amplification of the coagulative cascade. An essential role in the activation of blood coagulation is the tissue factor (TF), which triggers blood coagulation. The TF is found abundantly in the subendothelial collagen and is also expressed by fibroblasts providing a haemostatic covering layer ready to activate coagulation when the endothelial injury occurs. The aim of this review is to focus the attention on the underlying mechanisms related to haemostasis and thrombosis pathophysiology which may have a relevant role in SSc as well as on a possible role of anticoagulation in this disease.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Esclerodermia Sistémica
/
Trombosis
Límite:
Humans
Idioma:
En
Revista:
Clin Exp Med
Asunto de la revista:
MEDICINA
Año:
2023
Tipo del documento:
Article
País de afiliación:
Italia