Autoimmune Diseases Affecting Hemostasis: A Narrative Review

Int J Mol Sci. 2022 Nov 25;23(23):14715. doi: 10.3390/ijms232314715.

Abstract

Hemostasis reflects a homeostatic mechanism that aims to balance out pro-coagulant and anti-coagulant forces to maintain blood flow within the circulation. Simplistically, a relative excess of procoagulant forces can lead to thrombosis, and a relative excess of anticoagulant forces can lead to bleeding. There are a wide variety of congenital disorders associated with bleeding or thrombosis. In addition, there exist a vast array of autoimmune diseases that can also lead to either bleeding or thrombosis. For example, autoantibodies generated against clotting factors can lead to bleeding, of which acquired hemophilia A is the most common. As another example, autoimmune-mediated antibodies against phospholipids can generate a prothrombotic milieu in a condition known as antiphospholipid (antibody) syndrome (APS). Moreover, there exist various autoimmunity promoting environments that can lead to a variety of antibodies that affect hemostasis. Coronavirus disease 2019 (COVID-19) represents perhaps the contemporary example of such a state, with potential development of a kaleidoscope of such antibodies that primarily drive thrombosis, but may also lead to bleeding on rarer occasions. We provide here a narrative review to discuss the interaction between various autoimmune diseases and hemostasis.

Keywords: COVID-19; acquired hemophilia; antiphospholipid (antibody) syndrome (APS); autoimmune disease; bleeding; hemostasis; heparin induced thrombotic thrombocytopenia; immune thrombocytopenia; immune thrombotic thrombocytopenia; lupus anticoagulant; thrombosis; vaccine induced (immune) thrombotic thrombocytopenia.

Publication types

  • Review

MeSH terms

  • Anticoagulants
  • Antiphospholipid Syndrome*
  • Autoantibodies
  • COVID-19* / complications
  • Hemorrhage / complications
  • Hemostasis
  • Humans
  • Thrombosis* / complications

Substances

  • Anticoagulants
  • Autoantibodies

Grants and funding

This research received no external funding.