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

  • Antiphospholipid Syndrome (S Zuily, Section Editor)
  • Published:
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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.

Summary

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.

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Authors and Affiliations

  1. Division of Rheumatology, Hospital for Special Surgery, Weill Cornell Medical College, 535 East 70th Street, New York, NY, 10021, USA

    Salma Siddique

  2. CHRU de Nancy, Vascular Medicine Division and Regional Competence Center For Rare Vascular And Systemic Autoimmune Diseases, Inserm U1116 at Lorraine University, Nancy, France

    Jessie Risse & Stéphane Zuily

  3. Université Paris Descartes, Sorbonne Paris Cité; Inserm U1151, Institut Necker-Enfants Malades; Service de Néphrologie Transplantation Adultes, Hôpital Necker-Enfants Malades, Paris, France

    Guillaume Canaud

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  1. Salma Siddique
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  3. Guillaume Canaud
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  4. Stéphane Zuily
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Siddique, S., Risse, J., Canaud, G. et al. Vascular Manifestations in Antiphospholipid Syndrome (APS): Is APS a Thrombophilia or a Vasculopathy?. Curr Rheumatol Rep 19, 64 (2017). https://doi.org/10.1007/s11926-017-0687-z

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