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Increased Prevalence of Autonomic Dysfunction due to Postural Orthostatic Tachycardia Syndrome in Patients with Eosinophilic Gastrointestinal Disorders

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Kevin Z. Huang, Evan S. Dellon

Center for Esophageal Diseases and Swallowing, Center for Gastrointestinal Biology and Disease, Division of Gastroenterology and
Hepatology, University of North Carolina School of Medicine, Chapel Hill, NC, USA

DOI: http://dx.doi.org/10.15403/jgld.2014.1121.281.syd

Background & Aims: Eosinophilic gastrointestinal disorders (EGIDs) have been linked with atopic conditions, connective tissue disorders, and autoimmunity, but the association between EGIDs and autonomic dysfunction has not been extensively described. We aimed to assess this association and determine the prevalence of autonomic dysfunction in our EGID population.
Methods: In this retrospective cohort study, we analyzed adult patients in the University of North Carolina Eosinophilic Esophagitis (EoE)/EGID database who also had a diagnosis of comorbid autonomic dysfunction (postural orthostatic tachycardia syndrome [POTS] or gastroparesis). The electronic medical record was abstracted and disease characteristics summarized.
Results: We identified seven patients who had EGID and autonomic dysfunction. All had POTS and were Caucasian, and five (71%) were female; 2 also had gastroparesis. Onset of autonomic symptoms coincided with onset of EGID symptoms in 3 patients (43%) or with an acute EGID flare in 3. In no cases did successful control of EGID lead to improvement of autonomic symptoms. Two subjects (29%) had comorbid Ehlers- Danlos syndrome and mast cell activation syndrome. The prevalence of POTS in EGID patients was 7/560 (1.25%), compared to an estimated 0.17% prevalence of POTS in the general population.
Conclusions: The prevalence of autonomic dysfunction was 5-10-fold higher in EGID than expected in the general population, suggesting an association between the two conditions, though the underlying mechanism is unclear. Providers should consider comorbid autonomic dysfunction in patients with EGID, particularly when symptoms persist despite treatment.
Key words: eosinophilic gastrointestinal disorder – autonomic dysfunction – postural orthostatic tachycardia syndrome.
Abbreviations: CTD: connective tissue disorder; EC: eosinophilic colitis; EDS: Ehlers Danlos Syndrome; EoE: eosinophilic esophagitis; EG: eosinophilic gastritis; EGE: eosinophilic gastroenteritis;; EGID: eosinophilic gastrointestinal diseases; GI: gastrointestinal; IBD: inflammatory bowel disease; IBS: irritable bowel syndrome; IRB: institutional review board; MCAS: mast cell activation syndrome; POTS: postural orthostatic tachycardia
syndrome; TGF: transforming growth factor; TRPV1: transient receptor potential vanilloid type 1.