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

Abstract

<jats:p>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 &gt; 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.</jats:p>

Outcomes of Interdisciplinary Pain Rehabilitation Across Subgroups of the Multidimensional Pain Inventory – A Study From the Swedish Quality Registry for Pain Rehabilitation

“…Detailed reports on the psychometric properties of these variables have been presented in previous publications. 8,11,[40][41][42] In the SQRP, predetermined rules are used when handling single missing items of a scale or a subscale; details are reported elsewhere. 43 Baseline SQRP variables were used as descriptors of the clinical presentation at baseline together with background variables; several of these variables (as will be indicated) have been presented in a previous study.…”

“…43 Baseline SQRP variables were used as descriptors of the clinical presentation at baseline together with background variables; several of these variables (as will be indicated) have been presented in a previous study. 42 The Multidimensional Pain Inventory. The Swedish version of MPI was used.…”

Overlap between irritable bowel syndrome and hypermobileEhlers–Danlossyndrome: An unexplored clinical phenotype?

“…Central sensitization has been demonstrated in hEDS in the form of widespread hyperalgesia, including generalized chronic pain disorders occurring in 90% of patients (Molander et al, 2020), chronic headaches, and concomitant neck pain occurring in 53% of patients with migraine reported as the most common type of headache (Malhotra et al, 2020), temporomandibular joint disorder, occurring in 70% of patients (Hirsch, John, & Stang, 2008), and fibromyalgia occurring in 47% of patients (Sendur, Gurer, & Bozbas, 2007). Additionally, evidence of somatic sensitivity existing in those with overlap hEDS/HSD and FGID has been demonstrated using the PHQ-15 questionnaire, with Lam et al (2021) establishing that those with overlap hEDS/HSD and functional GI disorders have a significantly higher PHQ-15 somatization score compared to normal population controls.…”

Pain Symptomatology and Management in Pediatric Ehlers–Danlos Syndrome: A Review

“…There are numerous causes of pain in EDS, including hypermobility of joints, increased frequency of subluxation and dislocations of major and minor joints, injury to soft tissue and muscle pain, and prior surgical intervention [8]. Often, pain in EDS becomes chronic [9,10], leading to increased functional disability [3] and poorer psychological health [11] in adult patients [12].…”