Ehlers-Danlos Syndrome Associated With Various Complications in Hospitalized Patients

Steve Cimino

January 13, 2021

Hospitalized patients with Ehlers-Danlos syndrome (EDS) are more likely to have gastrointestinal, cardiovascular, autonomic, and allergic disorders than are hospitalized patients who do not have EDS, according to a new study of hospital outcomes in these four areas.

"Further research is necessary to explore the prevalence of these manifestations in the different subtypes of EDS and in outpatient population," wrote Rachel S. Brooks of the University of Connecticut, Farmington, and her coauthors. The study was published in Rheumatology.

To investigate previously observed connections between EDS and these four types of complications, the researchers launched a case-control study using hospital records from the 2016 National Inpatient Sample. A total of 2,007 patients with EDS were identified via ICD-10 code and matched with 4,014 non-EDS patients according to 5-year age intervals, sex, and month of admission. EDS patients had an average age of nearly 37, and 84% were female. The average hospitalization was lengthier for EDS patients (4.77 days) than for controls (4.07 days).

GI conditions were found in 44% of EDS patients, compared with 18% of controls (odds ratio, 3.57; 95% confidence interval, 3.17-4.02; P < .0001). Among the more likely conditions were functional disorders of the stomach (OR, 5.18; 95% CI, 2.16-12.42; P < .0001), unspecified abdominal pain (OR, 3.97; 95% CI, 2.34-6.73; P < .0001), irritable bowel syndrome (OR, 7.44; 95% CI, 5.07-10.94; P < .0001), and nausea (OR, 3.20; 95% CI, 1.95-5.24; P < .0001).

Autonomic dysfunction was found in 20% of EDS patients, compared with 6% of controls (OR, 4.45; 95% CI, 3.71-5.32; P < .0001). They were significantly more likely to have postural orthostatic tachycardia syndrome (OR, 223.77; 95% CI, 31.21-1604.46; P < .0001), orthostatic hypotension (OR, 8.98; 95% CI, 5.36-15.03; P < .0001), syncope (OR, 3.62; 95% CI, 2.23-5.82; P < .0001), and other autonomic nervous system disorders (OR, 54.72; 95% CI, 7.43-403.00; P < .0001).

Food allergies were also considerably more likely to occur in EDS patients (OR, 3.88; 95% CI, 2.65-5.66; P < .0001), as were cardiovascular complications like mitral valve disorders, aortic aneurysm, and cardiac dysrhythmias (OR, 6.16; 95% CI, 4.60-8.23; P < .0001). Although EDS patients were more likely to have hospital stays that lasted longer than 4 days, there was no notable difference in mortality (OR, 0.79; 95% CI, 0.41-1.50; P = .47).

After multivariate regression analysis that adjusted for age, sex, race, and smoking status, EDS patients were more likely to have GI (OR, 3.53; 95% CI, 3.08-4.03; P < .0001), autonomic (OR, 4.13; 95% CI, 3.40-5.01; P < .0001), allergic (OR, 3.92; 95% CI, 2.57-5.98; P < .0001), and cardiovascular complications (OR, 5.82; 95% CI, 4.21-8.03; P < .0001).

Shining a Much-Needed Light on the Conditions Associated With EDS

"Anyone who takes care of patients with EDS has likely seen some of these complications before and knows they can occur," Jordan T. Jones, DO, a pediatric rheumatologist at Children's Mercy Hospital in Kansas City, Mo., said in an interview. "I think this study legitimizes what many who take care of patients with EDS know to be true, and for those who don't, it brings a lot of attention to many of the symptoms and associated conditions."

He did, however, draw a conclusion that differed from one of the researchers' chief observations.

"They note that these patients have a longer-than-average hospital stay, suggesting that EDS may be linked to adverse complications during hospitalization," he said. "I think the reason for longer-than-average hospital stays is due to the number of symptoms and complexity of these patients, which can lead to delays in diagnosis. The complexity can lead to more involved evaluation that keeps them in the hospital longer than usual. Another reason for longer-than-average hospital stays that I've seen is the presentation of severe and chronic pain, which can be difficult to treat in the hospital and then transition to outpatient therapy. An inpatient hospitalization is not always the best place to treat chronic pain symptoms, which can drag out a hospital stay."

He also highlighted the lack of discussion regarding musculoskeletal complications, which he sees as one of the most common symptoms related to EDS.

"As a rheumatologist, I see many patients with EDS present with chronic pain, chronic muscle weakness, and chronic fatigue. If you think about the joint laxity with EDS, these patients are a perfect setup to develop tight, weak muscles, which leads to a lot of musculoskeletal pain and fatigue."

That said, he ultimately emphasized the clear benefits of such a large study on such an under-researched subject.

"We think EDS is more common than is reported," he said. "But despite that, there are still a lot of people who don't know about EDS, understand it, or appreciate how to evaluate for it. One of the best things this study does is bring more visibility to this disease and the associated conditions related to it."

The authors declared no potential conflicts of interest.

SOURCE: Brooks RS et al. Rheumatology. 2021 Jan 7. doi: 10.1093/rheumatology/keaa926.

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