Vascular Manifestations in Antiphospholipid Syndrome (APS): Is APS a Thrombophilia or a Vasculopathy?

Curr Rheumatol Rep. 2017 Sep 4;19(10):64. doi: 10.1007/s11926-017-0687-z.

Abstract

Purpose of review: Antiphospholipid antibody syndrome (APS) is characterized primarily by thrombosis and pregnancy morbidity. Chronic vascular lesions can also occur. While the underlying mechanisms of these vascular lesions are not entirely known, there have been multiple theories describing the potential process of vasculopathy in APS and the various clinical manifestations associated with it.

Recent findings: Recently, it has been demonstrated that endothelial proliferation in kidneys can be explained by the activation of the mammalian target of rapamycin complex (mTORC) pathway by antiphospholipid antibodies (aPL). These data support the existence of an APS-related vasculopathy in different locations which can explain-in part-the different manifestations of APS. This review focuses on the various manifestations of APS as a result of APS-related vasculopathy, as well as pathophysiology, current screening, and treatment options for clinicians to be aware of.

Keywords: Antiphospholipid syndrome; Endothelium; Proliferation; Sirolimus; Smooth muscle cells; Vasculopathy.

Publication types

  • Review

MeSH terms

  • Antiphospholipid Syndrome / complications
  • Antiphospholipid Syndrome / diagnosis
  • Antiphospholipid Syndrome / drug therapy
  • Antiphospholipid Syndrome / physiopathology*
  • Humans
  • Thrombophilia / physiopathology
  • Vascular Diseases / diagnosis
  • Vascular Diseases / drug therapy
  • Vascular Diseases / etiology
  • Vascular Diseases / physiopathology*
  • Vasculitis / physiopathology