Disclosures: The researchers report no relevant financial disclosures.
January 26, 2021
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GI, cardiovascular, autonomic, allergic symptoms more prevalent in Ehlers-Danlos syndrome

Disclosures: The researchers report no relevant financial disclosures.
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Patients with Ehlers-Danlos syndrome experience significantly more prevalent gastrointestinal, cardiovascular, autonomic and allergic symptoms than those in the hospital without the disease, according to data published in Rheumatology.

“Previous studies suggest associations between EDS and gastrointestinal, cardiovascular, immune, and autonomic nervous system dysfunction, but no large case-control studies have examined the prevalence of these conditions in hospitalized EDS patients to date,” Rachel S. Brooks, of the University of Connecticut, and colleagues wrote. “Most studies reporting gastrointestinal abnormalities and autonomic dysfunction in EDS have used small sample sizes or observations from specialty clinics.”

Patients with Ehlers-Danlos syndrome experience significantly more prevalent gastrointestinal, cardiovascular, autonomic and allergic symptoms than those in the hospital without the disease, according to data.

“Case reports demonstrate associations between cardiovascular complications and EDS, and one study finding eosinophilic esophagitis in patients with hereditary connective tissue disorders has suggested a link between food allergies and EDS,” they added. “Because these associations have not been further examined, the true frequency and nature of these conditions in EDS remains unknown.”

To examine whether a hospital diagnosis of Ehlers-Danlos syndrome is associated with a higher prevalence of these manifestations and complications, compared with patients in the hospital without the disease, Brooks and colleagues analyzed data from the 2016 National Inpatient Sample. Part of the Healthcare Cost and Utilization Project, this sample collects a 20% stratified sample of discharges from participating hospitals each year, amassing records on more than 7 million stays annually.

In all, the researchers identified 2,007 cases of Ehlers-Danlos syndrome using ICD-10 codes. These cases were matched 1:2 to 4,014 control patients without Ehlers-Danlos syndrome, based on 5-year age intervals, sex, month of admission. The primary outcome was the prevalence of gastrointestinal manifestations, autonomic dysfunction, food allergies and cardiovascular complications, compared between the two groups. The researchers performed bivariate analyses, as well as multivariate logistic regression models to adjust for confounders.

According to the researchers, 44% of patients with Ehlers-Danlos syndrome demonstrated gastrointestinal manifestations, compared with 18% of control individuals (OR = 3.57; 95% CI, 3.17-4.02). In addition, irritable bowel syndrome, gastroparesis and celiac disease were strongly associated with Ehlers-Danlos syndrome.

Autonomic dysfunction, meanwhile, including postural orthostatic tachycardia syndrome, neurocardiogenic syncope and orthostatic hypotension, was recorded in 20% of patients with Ehlers-Danlos syndrome, compared with 6% of control individuals (OR = 4.45; 95% CI, 3.71-5.32)

Patients with Ehlers-Danlos syndrome were also more likely to demonstrate food allergies (OR=3.88; 05% CI, 2.65-5.66) and cardiovascular complications, including mitral valve disorders, aortic aneurysm and dysrhythmias (OR=6.16; CI, 4.6-8.23).

These conditions remained highly associated with the disease after adjusting for confounders. In addition, patients with Ehlers-Danlos syndrome were 76% more likely to experience longer-than-average hospitalizations (OR=1.76; CI, 1.54-2.02).

“This large case-control study of hospitalized patients determined that a diagnosis of EDS is associated with a higher prevalence of gastrointestinal, cardiovascular, autonomic, and allergic manifestations versus hospitalized patients without EDS,” Brooks and colleagues wrote. “Patients with EDS are more likely to have longer-than-average hospital stays, suggesting that EDS may be linked to adverse complications during hospitalization.”