Abstract
Introduction
It is not clear if patients with postural tachycardia syndrome (POTS) and Ehlers-Danlos
syndrome (hEDS) differ from patients with POTS due to other etiologies. We compared
the results of autonomic testing and healthcare utilization in POTS patients with
and without hEDS.
Methods
Patients with POTS + hEDS (n = 20) and POTS controls without hypermobility (n = 20) were included in the study. All patients underwent autonomic testing, and the
electronic medical records were reviewed to determine the number and types of medications
patients were taking, as well as the number of outpatient, emergency department, and
inpatient visits over the prior year.
Results
Patients with hEDS had twice as many outpatient visits (21 v. 10, p = 0.012), were taking more prescription medications (8 vs. 5.5, p = 0.030), and were more likely to see a pain physician (70% vs 25%, p = 0.005). Autonomic testing demonstrated a slight reduction in heart rate variability
and slightly lower blood pressures on tilt table testing in hEDS patients, however
for most patients these variables remained within the range of normal. Orthostatic
tachycardia on tilt table testing was greater in POTS controls (46 bpm vs 39 bpm, p = 0.018). Abnormal QSweat responses were common in both groups (38% of POTS + hEDS and 36% of POTS controls).
Conclusions
While autonomic testing results were not significantly different between groups, patients
with POTS + hEDS took more medications and had greater markers of healthcare utilization, with
chronic pain likely playing a prominent role.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Autonomic Neuroscience: Basic and ClinicalAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Gastrointestinal disorders in joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type: a review for the gastroenterologist.Neurogastroenterol. Motil. 2016;
- Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK).Am. J. Med. Genet. 1998; 77: 31-37
- Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes.Neurology. 2016; 87: 155-159
- An association between anxiety and neurocardiogenic syncope during head-up tilt table testing.Pacing Clin. Electrophysiol. 2000; 23: 837-841
- Painful small fiber neuropathy with gastroparesis: a new phenotype with a novel mutation in the SCN10A gene.J. Clin. Neurosci. 2016; 26: 84-88
- Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers–Danlos syndrome.Semin. Arthritis Rheum. 2014; 44: 93-100
- Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome.Arthritis Rheum. 2004; 50: 3323-3328
- Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome.Auton. Neurosci. 2011; 161: 46-48
- Dysautonomia in the joint hypermobility syndrome.Am. J. Med. 2003; 115: 33-40
- Joint hypermobility and skin elasticity: the hereditary disorders of connective tissue.Clin. Dermatol. 2006; 24: 521-533
- Connective tissue disorders in patients with spontaneous intracranial hypotension.Cephalalgia. 2011; 31: 691-695
Low, Phillip A., Sletten DM. 2008. In: Clinical Autonomic Disorders, 3rd ed. Philadelphia, PA, p 130–163.
- Deconditioning in patients with orthostatic intolerance.Neurology. 2012; 79: 1435-1439
- Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study.Eur. J. Hum. Genet. 2013; 21: 386-390
- Epidemiology of general joint hypermobility and basis for the proposed criteria for benign joint hypermobility syndrome: review of the literature.J. Rheumatol. 2007; 34: 804-809
- Diagnostic criteria for headache due to spontaneous intracranial hypotension: a perspective.Headache. 2011; 51: 1442-1444
- Normative values for sudomotor axon reflex testing using QSWEAT™.Neurology. 2015; 84: P01.282
- The Ehlers-Danlos syndrome.in: Connective Tissue and Its Heritable Disorders. John Wiley & Sons, Inc., Hoboken, NJ, USA2002: 431-523
- Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome Type III and Ehlers-Danlos syndrome hypermobility type): clinical description and natural history.Am. J. Med. Genet. C Semin. Med. Genet. 2017; 175: 48-69
- Recurrent neuropathy associated with Ehlers–Danlos syndrome.J. Neurol. 2006; 253: 670-671
- Neuromuscular involvement in various types of Ehlers-Danlos syndrome.Ann. Neurol. 2009; 65: 687-697
- Ehlers-Danlos Syndrome and Postural Tachycardia Syndrome: a relationship study.J. Neurol. Sci. 2014; 15: 99-102
- Unexplained gastrointestinal symptoms and joint hypermobility: is connective tissue the missing link?.Neurogastroenterol. Motil. 2010; 22252-e78
Article Info
Publication History
Published online: October 02, 2017
Accepted:
October 1,
2017
Received in revised form:
September 8,
2017
Received:
June 13,
2017
Identification
Copyright
© 2017 Elsevier B.V. All rights reserved.
