Delineating the deranged immune system in the antiphospholipid syndrome

Area of Science:

• Immunology
• Autoimmune Diseases
• Systems Medicine

Background:

• Antiphospholipid syndrome (APS) is a systemic autoimmune disorder.
• Characterized by antiphospholipid antibodies (aPL), vascular thrombosis, and obstetric complications.
• Beta-2 glycoprotein I (β2GPI) is a key autoantigen targeted in APS.

Purpose of the Study:

• To review genetic and environmental factors in APS.
• To summarize current knowledge on immune system abnormalities in APS.
• To identify gaps in understanding APS pathophysiology and propose future research directions.

Main Methods:

• Literature review of genetic and environmental factors.
• Summary of current research on innate and adaptive immune system components in APS.
• Discussion of the roles of various immune cells (dendritic cells, T-cells, B-cells, monocytes, neutrophils, NK-cells) and the complement system.

Main Results:

• Anti-β2GPI antibodies activate endothelial cells, thrombocytes, and placental tissue, linking to APS clinical manifestations.
• Abnormalities in both innate and adaptive immunity are implicated in APS.
• Several knowledge gaps exist regarding the detailed pathophysiology of APS.

Conclusions:

• Further research is needed to fully elucidate the pathogenesis of APS.
• Extensive immune cell profiling using a systems medicine approach is recommended.
• This approach may reveal novel biomarkers and therapeutic targets for APS.