Postural tachycardia syndrome (POTS) and antiphospholipid syndrome (APS): What do we know so far? - 09/05/22

Doi : 10.1016/j.neurol.2021.10.006

H.A. Noureldine ^a, G. El Hasbani ^b, M. Nour Eldine ^a, W. Nour-Eldine ^c, A. Taher ^d, I. Uthman ^b,^⁎
^a Gilbert and Rose-Marie Chagoury School of Medicine, Lebanese American University, Byblos, Lebanon
^b Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon
^c Neurological Disorders Research Center, Qatar Biomedical Research Institute, Hamad Bin Khalifa University, Qatar foundation, Doha, Qatar
^d Division of Hematology and Medical Oncology, Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon

^⁎Corresponding author. Division of Rheumatology, American University of Beirut Medical Center, Riad El-Solh, P.O. Box 11-0236, Beirut 1107 2020, Lebanon.Division of Rheumatology, American University of Beirut Medical CenterRiad El-Solh, P.O. Box 11-0236Beirut1107 2020Lebanon

Highlights

•	Several connective tissue diseases, recently APS, have been associated with POTS.
•	Certain pathophysiological mechanisms including stress, infections and autoantibodies are common in both diseases and might help explain the association between them.
•	Further studies are needed to decide whether POTS and other autonomic disorders are to be added to the international criteria of APS.

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Abstract

As part of the non-criteria clinical manifestations, postural orthostatic tachycardia syndrome (POTS), a multisystem autonomic dysfunction, can co-exist with antiphospholipid syndrome (APS). Several pieces of evidence hint on the autoimmune basis of POTS, and its possible association with several autoimmune diseases, including APS. Indeed, the evidence exists in the etiologies, symptomatology, and treatment options. Although infections, viral ones in particular, stress, and pregnancy are etiologies to both POTS and APS, the exact pathophysiological connection is still to be studied taking into consideration the activity of cytokines in both diseases. Nevertheless, certain immunomodulatory treatments used for the catastrophic or obstetrical forms of APS, such as intravenous immunoglobulins (IVIG) and steroids, have been also used for the treatment of POTS resistant to classical treatments. Therefore, our review aims to highlight the association between POTS and APS, shedding light on the common etiologies explaining the pathophysiology of the two disorders, the diagnostic approach to POTS as a possible clinical criterion of APS, and the treatment of APS in the context of treating POTS.

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Keywords : Antiphospholipid syndrome, Postural orthostatic tachycardia syndrome, Autonomic dysfunction, Antiphospholipid antibodies, Cytokines, Anticoagulation