title: Postural tachycardia syndrome (POTS) and other autonomic disorders in antiphospholipid (Hughes) syndrome (APS).
abstract: BACKGROUND
  Antiphospholipid syndrome (APS) is an autoimmune hypercoagulable disorder that has been shown to cause a large number of cardiac and neurological manifestations. Two recent studies have demonstrated abnormalities in cardiovascular autonomic function testing in APS patients without other cardiovascular or autoimmune disease. However, an association between autonomic disorders such as postural tachycardia syndrome and APS has not previously been described.
  
  
  RESULTS
  Data were obtained by retrospective chart review. We identified 15 patients who have been diagnosed with APS and an autonomic disorder. The median age of the patients at the time of data analysis was 39 years. The autonomic disorders seen in these patients included postural tachycardia syndrome, neurocardiogenic syncope and orthostatic hypotension. The majority of patients (14/15) were female and the majority (14/15) had non-thrombotic neurological manifestations of APS, most commonly migraine, memory loss and balance disorder. Many also had livedo reticularis (11/15) and Raynaud's phenomenon (nine of 15). In some patients, the autonomic manifestations improved with anticoagulation and/or anti-platelet therapy; in others they did not. Two patients with postural tachycardia syndrome who failed to improve with the usual treatment of APS have been treated with intravenous immunoglobulin with significant improvement in their autonomic symptoms.
  
  
  CONCLUSIONS
  We believe that autonomic disorders in APS may represent an important clinical association with significant implications for treatment.
authors.affiliation: Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA.
authors.forename: J R
authors.initials: JR
authors.lastname: Schofield
authors.affiliation: Department of Neurology, State University at Buffalo School of Medicine and Biomedical Sciences, Buffalo, NY, USA.
authors.forename: S
authors.initials: S
authors.lastname: Blitshteyn
authors.affiliation: Sackler Faculty in Medicine, Laura Schwarz-Kipp Chair for Research of Autoimmune Diseases, Tel-Aviv University, Israel Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel.
authors.forename: Y
authors.initials: Y
authors.lastname: Shoenfeld
authors.affiliation: The London Lupus Centre, London Bridge Hospital, London, UK Graham.Hughes@HCAConsultant.co.uk.
authors.forename: G R V
authors.initials: GR
authors.lastname: Hughes
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016736:Antiphospholipid Syndrome / Q000150:complications*; D001342:Autonomic Nervous System Diseases / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D054972:Postural Orthostatic Tachycardia Syndrome / Q000209:etiology*; D012189:Retrospective Studies; D055815:Young Adult
keywords: Hughes syndrome; antiphospholipid syndrome (APS); autonomic disorders; intravenous immunoglobulin (IVIG); neurocardiogenic syncope (NCS); postural tachycardia syndrome (POTS)