Lupus (2018) 0, 1–10 journals.sagepub.com/home/lup REVIEW Cardiovascular reflex tests in patients with systemic lupus erythematosus: clinical performance and utility PS Matusik 1 , PT Matusik 2,3 and PK Stein 4 1 Department of Radiology, University Hospital, Krako´ w, Poland; 2 Department of Electrocardiology, The John Paul II Hospital, Krako´ w, Poland; 3 Institute of Cardiology, Faculty of Medicine, Jagiellonian University Medical College, Krako´w, Poland; and 4 Department of Medicine, Cardiovascular Division, Washington University School of Medicine, St. Louis, MO, USA Background: This review summarizes current knowledge about cardiovascular reflex tests (CVRTs) and other selected autonomic nervous system (ANS) assessment tests in systemic lupus erythematosus (SLE) patients and assesses their clinical utility in this group of patients. Methods: The PubMed database was searched for terms associated with CVRTs and SLE. Only papers available in full text and published in English were considered. Ultimately, 13 were selected and analyzed. Results: In most of the studies CVRTs results were reported more likely to be abnormal in patients with SLE when compared with controls. The reported prevalence of ANS dysfunction in SLE, diagnosed using CVRTs, ranged from 23.5% to 82.7% of patients, likely because of different definitions of ANS dysfunction, vari- ability in methods of performing CVRTs, and potential confounding factors. In general CVRTs results did not correlate with SLE activity or disease duration, but some CVRTs results correlated with some peptides associated with ANS function, including neuropeptide Y and vasoactive intestinal peptide. Conclusion: Patients with SLE generally have abnormal or borderline results of CVRTs, which indicate prevalent abnormalities of the ANS in SLE. Performance of CVRTs requires good standardization of test conditions and familiarity with the proper administration and interpretation of these tests. Lupus (2018) 0, 1–10. Key words: Systemic lupus erythematosus; cardiovascular reflex tests; autonomic nervous system dysfunction Introduction Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of the connective tissue, char- acterized by varying degrees of severity and a heterogeneous natural course. 1 Inflammation, vas- culitis, immune complex deposition, and vasculopa- thy are involved in the pathogenesis of SLE. 1,2 SLE can affect almost any organ system, including the autonomic nervous system (ANS). 3 The pathogen- esis of the ANS dysfunction in patients with SLE is still unclear, although it appears to have an auto- immune component. 4–6 SLE presents with a wide range of symptoms and signs. 7–13 Some of them are nonspecific (e.g. weakness, dizziness, lightheaded- ness, syncope, and fatigue) and may be a result of ANS dysfunction. Therefore, it might be useful to assess ANS involvement per se in patients with SLE. 14,15 Cardiovascular reflex tests (CVRTs) are a well- known, noninvasive technique for measurement of ANS function. CVRTs are based on an assessment of reflex changes in heart rate (HR) and blood pres- sure (BP) in response to standardized stimuli, including deep breathing, the Valsalva maneuver, lying down to standing up and handgrip. 6 A stand- ard battery of CVRTs was described by Ewing and Clarke in 1982 and consists of five tests performed in the following sequence: HR response to Valsalva maneuver, HR response to deep breathing, BP response to sustained handgrip, HR response to standing, and BP response to standing. 16 Results of particular CVRTs are then categorized as normal (scored as 0), borderline (1) or abnormal (2). Ewing and Clarke defined CVRT-assessed ANS function as normal (all five tests normal or one borderline), early (when one of the three HR Correspondence to: Phyllis K. Stein, Department of Medicine, Cardiovascular Division, Washington University School of Medicine, 660 South Euclid Avenue, St. Louis, MO 63110-1010, USA. Email: pstein@wustl.edu Received 10 April 2018; accepted 23 May 2018 ! The Author(s), 2018. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav 10.1177/0961203318783052