Ehlers-Danlos Syndrome and Hypermobility Syndrome Compared with Other Common Chronic Pain Diagnoses-A Study from the Swedish Quality Registry for Pain Rehabilitation.

Peter Molander,Monika Löfgren,Andrea Hållstam,Britt-Marie Stålnacke,Mehmed Novo,Björn Gerdle

doi:10.3390/jcm9072143

Abstract

Although chronic pain is common in patients with Ehlers–Danlos syndrome (EDS) and hypermobility syndromes (HMS), little is known about the clinical characteristics of these groups. The main aim was to compare EDS/HMS with common local and generalized pain conditions with respect to Patient Reported Outcome Measures (PROMs). Data from the Swedish Quality Register for Chronic Pain (SQRP) from 2007 to 2016 (n = 40,518) were used, including patients with EDS/HMS (n = 795), fibromyalgia (n = 5791), spinal pain (n = 6693), and whiplash associated disorders (WAD) (n = 1229). No important differences in the PROMs were found between EDS and HMS. Women were represented in > 90% of EDS/HMS cases and fibromyalgia cases, and in about 64% of the other groups. The EDS/HMS group was significantly younger than the others but had a longer pain duration. The pain intensity in EDS/HMS was like those found in spinal pain and WAD; fibromyalgia had the highest pain intensity. Depressive and anxiety symptoms were very similar in the four groups. Vitality—a proxy for fatigue—was low both in EDS/HMS and fibromyalgia. The physical health was lower in EDS/HMS and fibromyalgia than in the two other groups. Patients with EDS/HMS were younger, more often female, and suffered from pain for the longest time compared with patients who had localized/regional pain conditions. Health-care clinicians must be aware of these issues related to EDS/HMS both when assessing the clinical presentations and planning treatment and rehabilitation interventions.

Highlights

Ehlers–Danlos syndrome (EDS) is a heritable condition characterized by a disorder in the collagen, causing increased laxity in bodily organs such as skin, ligament, joints, blood vessels, and inner organs [1]
Since pain is a criterion for EDS hypermobility type we assumed that most of those patients belonged to this sub-type
As there was a potential for lack of precision in differential diagnosis between these related diagnoses, we looked for any systematic differences between these groups on the measures included in the study

Summary

Introduction

Ehlers–Danlos syndrome (EDS) is a heritable condition characterized by a disorder in the collagen, causing increased laxity in bodily organs such as skin, ligament, joints, blood vessels, and inner organs [1]. Generalized hypermobility in childhood increases the risk of joint pain during teenage years [6]. Joint hypermobility is often associated with daily pain and increases with age, negatively affecting the physical function of young people [6,7]. A Numeric Rating Scale (NRS) was used to rate average pain intensity for the last week, with a possible score from 0 to 10 where the highest number represents worst possible pain. Female gender was more common for EDS/HMS (93.6%) and fibromyalgia (95.1%) (Table 3). WAD had the highest proportion with high education (29.1%) and EDS/HMS (25.5%), whereas patients with fibromyalgia had the lowest proportion (19.8%) (Table 3). Working full time had the following distribution: WAD (50.4%), spinal pain (46.7%), EDS/HMS (46.2%), and fibromyalgia (38.7%) (Table 3).

Results

Discussion

Conclusion