Salma Siddique1, Jessie Risse2, Guillaume Canaud3, Stéphane Zuily2. 1. Division of Rheumatology, Hospital for Special Surgery, Weill Cornell Medical College, 535 East 70th Street, New York, NY, 10021, USA. siddiques@hss.edu. 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. 3. Université Paris Descartes, Sorbonne Paris Cité; Inserm U1151, Institut Necker-Enfants Malades; Service de Néphrologie Transplantation Adultes, Hôpital Necker-Enfants Malades, Paris, France.
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.
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.
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Authors: Stéphane Zuily; Isabelle Clerc-Urmès; Cédric Bauman; Danieli Andrade; Savino Sciascia; Vittorio Pengo; Maria G Tektonidou; Amaia Ugarte; Maria Gerosa; H Michael Belmont; Maria Angeles Aguirre Zamorano; Paul Fortin; Lanlan Ji; Maria Efthymiou; Hannah Cohen; D Ware Branch; Guilherme Ramires de Jesus; Cecilia Nalli; Michelle Petri; Esther Rodriguez; Ricard Cervera; Jason S Knight; Tatsuya Atsumi; Rohan Willis; Maria Laura Bertolaccini; Joann Vega; Denis Wahl; Doruk Erkan Journal: Lupus Date: 2020-07-23 Impact factor: 2.911