High overlap in patients diagnosed with hypermobile Ehlers-Danlos syndrome or hypermobile spectrum disorders with fibromyalgia and 40 self-reported symptoms and comorbidities

Abstract

BackgroundJoint pain is a common symptom in patients with hypermobile Ehlers-Danlos Syndrome (hEDS), hypermobility spectrum disorders (HSD) and fibromyalgia. The goal of this study was to determine whether symptoms and comorbidities overlap in patients diagnosed with hEDS/HSD and/or fibromyalgia.MethodsWe retrospectively examined self-reported data from an EDS Clinic intake questionnaire in patients diagnosed with hEDS/HSD, fibromyalgia, or both vs. controls with an emphasis on joint issues.ResultsFrom 733 patients seen at the EDS Clinic, 56.5% (n = 414) were diagnosed with hEDS/HSD and fibromyalgia (Fibro), 23.8% (n = 167) hEDS/HSD, 13.3% (n = 98) fibromyalgia, or 7.4% (n = 54) none of these diagnoses. More patients were diagnosed with HSD (76.6%) than hEDS (23.4%). Patients were primarily White (95%) and female (90%) with a median age in their 30s (controls 36.7 [18.0, 70.0], fibromyalgia 39.7 [18.0, 75.0], hEDS/HSD 35.0 [18.0, 71.0], hEDS/HSD&Fibro 31.0 [18.0, 63.0]). There was high overlap in all 40 symptoms/comorbidities that we examined in patients diagnosed with fibromyalgia only or hEDS/HSD&Fibro, regardless of whether they had hEDS or HSD. Patients that only had hEDS/HSD without fibromyalgia had far fewer symptoms/comorbidities than patients with hEDS/HSD&Fibro. The top self-reported issues in patients that only had fibromyalgia were joint pain, hand pain when writing or typing, brain fog, joint pain keeping from daily activities, allergy/atopy and headache. Five issues that significantly and uniquely characterized patients diagnosed with hEDS/HSD&Fibro were subluxations (dislocations in hEDS patients), joint issues like sprains, the need to stop sports due to injuries, poor wound healing, and migraine.ConclusionThe majority of patients seen at the EDS Clinic had a diagnosis of hEDS/HSD plus fibromyalgia that was associated with more severe disease. Our findings indicate that fibromyalgia should be routinely assessed in patients with hEDS/HSD and vis-a-versa to improve patient care.

Cardiac defects of hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders: a retrospective cohort study

“…We found very few patients with hEDS or HSD that had a dilated aortic root in this study. Additionally, we did not find a significant difference in z-score between patients with hEDS or HSD, which is perhaps not surprising considering the high degree of overlap in many clinical features we and others have found between the two diagnoses ( 5 , 10 , 16 ). We were not using a pediatric z-score but one that incorporated body size area (BSA) as recently recommended for adults ( 12 , 13 ).…”

“…All patients seen at the EDS Clinic were evaluated by the same physician who is an EDS specialist and diagnosed according to the 2017 EDS diagnostic criteria ( 2 ). Adult patients received a diagnosis of hEDS or HSD, which we reported previously have a high overlap of symptoms and comorbidities ( 10 ). Patients that attended the EDS Clinic but did not obtain a diagnosis of hEDS or HSD were also examined.…”

“…Self-reported demographics, patient history and cardiac history were obtained from all patients who attended the EDS Clinic using a Research Electronic Data Capture (REDCap) Intake Questionnaire, as reported previously ( 10 ).…”

Phenotypic Clusters and Multimorbidity in Hypermobile Ehlers-Danlos Syndrome

“…Our institutional review board approved only inclusion of hEDS and not individuals with significant phenotypic overlap such as hypermobility spectrum disorder. 11 Data from individuals without an hEDS diagnosis (N=240) and those reporting that they have been diagnosed with another subtype of EDS (N=52) or another type of CTD (N=50) were excluded from the survey. This resulted in 2149 participants who answered all questions and were included in the study ( Figure 1 A).…”

Longitudinal cytokine and multi-modal health data of an extremely severe ME/CFS patient with HSD reveals insights into immunopathology, and disease severity

“…The inconsistency in research findings could be due to a myriad of factors including the dynamic nature of ME/CFS ( 10 ), characterized by relapse and remission cycles, diverse triggers, and variable durations, as well as medication, lifestyle, and comorbidities ( 31 , 35 , 45 – 48 ). Adding to the complexity is the subjective nature of symptomatology, spanning across multiple bodily systems, and the inherent limitations in tools available for the quantitative measurement of severity fluctuations.…”