Mechanisms of immunothrombosis and vasculopathy in antiphospholipid syndrome

• Published in: Seminars in immunopathology Vol. 44; no. 3; pp. 347 - 362
• Main Authors: Knight, Jason S., Kanthi, Yogendra
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.05.2022; Springer Nature B.V
• Subjects: Antibodies, Antiphospholipid; Antiphospholipid antibodies; Antiphospholipid syndrome; Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - diagnosis; Autoimmune diseases; Biomedical and Life Sciences; Biomedicine; Cell proliferation; Complement activation; COVID-19; COVID-19 - complications; Endothelial Cells; Fibrin; Humans; Immunology; Internal Medicine; Leukocytes (neutrophilic); Pathology; Phospholipids; Review; Thromboinflammation; Thrombophilia; Thrombosis; Thrombosis - complications; Vascular Diseases; Vasculopathy; Thrombosis; Neutrophil extracellular traps; Antiphospholipid syndrome
• ISSN: 1863-2297; 1863-2300; 1863-2300
• DOI: 10.1007/s00281-022-00916-w

Antiphospholipid syndrome (APS) is an autoimmune thrombophilia propelled by circulating antiphospholipid antibodies that herald vascular thrombosis and obstetrical complications. Antiphospholipid antibodies recognize phospholipids and phospholipid-binding proteins and are not only markers of disease but also key drivers of APS pathophysiology. Thrombotic events in APS can be attributed to various conspirators including activated endothelial cells, platelets, and myeloid-lineage cells, as well as derangements in coagulation and fibrinolytic systems. Furthermore, recent work has especially highlighted the role of neutrophil extracellular traps (NETs) and the complement system in APS thrombosis. Beyond acute thrombosis, patients with APS can also develop an occlusive vasculopathy, a long-term consequence of APS characterized by cell proliferation and infiltration that progressively expands the intima and leads to organ damage. This review will highlight known pathogenic factors in APS and will also briefly discuss similarities between APS and the thrombophilic coagulopathy of COVID-19.