Similarities and differences in self-reported symptoms and comorbidities between hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders.

IF 2.1 Q3 RHEUMATOLOGY Rheumatology Advances in Practice Pub Date : 2024-11-04 eCollection Date: 2024-01-01 DOI:10.1093/rap/rkae134
Ashley A Darakjian, Mira Bhutani, DeLisa Fairweather, S Christian Kocsis, Jessica J Fliess, Sami Khatib, Gabe J Weigel, Elizabeth J McCabe, Varsini Balamurugan, Evan E Perona, Jessica M Gehin, Emily R Whelan, Angita Jain, Hanna Sledge, David O Hodge, Todd D Rozen, Francis A Farraye, Ozan Soyer, Joseph Cheung, Stephanie L Grach, David Shirey, Shilpa Gajarawala, Bala Munipalli, Chrisandra L Shufelt, Dacre R T Knight, Katelyn A Bruno
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Abstract

Objectives: Patients with hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) experience a wide array of symptoms and system disorders. This study aimed to identify whether differences occurred in 115 self-reported symptoms and comorbidities in patients diagnosed with hEDS or HSD.

Methods: In this study we analysed self-reported data from an EDS Clinic intake questionnaire in patients diagnosed with hEDS, HSD or no hypermobile conditions.

Results: From 1 November 2019 to 7 March 2024, the EDS Clinic saw 2088 patients. Using the 2017 diagnostic criteria, 66.5% were diagnosed with HSD (n = 1389), 20.3% with hEDS (n = 423), 10.6% with historic HSD (H-HSD) or localized HSD (L-HSD) (n = 256) (hypermobile controls) and 2.6% were not diagnosed with hEDS, HSD, H-HSD or L-HSD (n = 55) (controls). Symptoms/comorbidities that occurred with high prevalence in both hEDS and HSD included joint pain (hEDS 82.0%, HSD 88.9%), allergy (hEDS 77.0%, HSD 77.0%), subluxations (hEDS 71.2%, HSD 72.6%), brain fog (hEDS 70.0%, HSD 74.7%), headache (hEDS 68.1%, HSD 69.1%), anxiety (hEDS 60.3%, HSD 69.3%), depression (hEDS 52.2%, HSD 58.0%), migraine (hEDS 53.7%, HSD 52.5%), nausea (hEDS 54.6%, HSD 59.5%) and constipation (hEDS 53.0%, HSD 57.2%). In contrast, 9/115 (8%) symptoms/comorbidities were self-reported significantly more often in hEDS but 42/115 (37%) in HSD. hEDS patients reported more symptoms that suggest a defect in collagen, such as dislocation, hernias and rectal prolapse, while HSD patients reported more joint, muscle, allergy, neurological, gastrointestinal, sleep and psychological symptoms/comorbidities.

Conclusion: Although we found an overlap in some symptoms and comorbidities self-reported by hEDS/HSD patients, such as allergy/atopy, headache/migraine and gastrointestinal symptoms, our findings suggest key differences exist between the two diagnoses, suggesting that hEDS and HSD may be distinct conditions.

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多动性ehers - danlos综合征与多动性谱系障碍自我报告症状和合并症的异同
目的:多动性ehers - danlos综合征(hEDS)和多动性谱系障碍(HSD)患者会经历一系列症状和系统障碍。本研究旨在确定115名被诊断为hEDS或HSD的患者自我报告的症状和合并症是否存在差异。方法:在这项研究中,我们分析了诊断为hEDS、HSD或无过度运动状况的患者在EDS诊所摄入问卷中自我报告的数据。结果:从2019年11月1日到2024年3月7日,EDS诊所接待了2088名患者。根据2017年的诊断标准,66.5%的患者被诊断为HSD (n = 1389), 20.3%的患者被诊断为hEDS (n = 423), 10.6%的患者被诊断为历史性HSD (H-HSD)或局限性HSD (L-HSD) (n = 256)(超运动对照组),2.6%的患者未被诊断为hEDS、HSD、H-HSD或L-HSD (n = 55)(对照组)。hds和HSD患病率较高的症状/合共包括关节痛(hds 82.0%, HSD 88.9%)、过敏(hds 77.0%, HSD 77.0%)、半脱位(hds 71.2%, HSD 72.6%)、脑烟雾(hds 70.0%, HSD 74.7%)、头痛(hds 68.1%, HSD 69.1%)、焦虑(hEDS 60.3%, HSD 69.3%)、抑郁(hEDS 52.2%, HSD 58.0%)、偏头痛(hEDS 53.7%, HSD 52.5%)、恶心(hEDS 54.6%, HSD 59.5%)和便秘(hEDS 53.0%, HSD 57.2%)。相比之下,9/115(8%)的hEDS患者自我报告的症状/合并症明显更多,而42/115(37%)的HSD患者自我报告的症状/合并症明显更多。hEDS患者报告了更多提示胶原蛋白缺陷的症状,如脱位、疝气和直肠脱垂,而HSD患者报告了更多的关节、肌肉、过敏、神经、胃肠、睡眠和心理症状/合并症。结论:虽然我们发现hEDS/HSD患者自我报告的一些症状和合并症有重叠,如过敏/特应性、头痛/偏头痛和胃肠道症状,但我们的研究结果表明两种诊断存在关键差异,表明hEDS和HSD可能是不同的疾病。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Rheumatology Advances in Practice
Rheumatology Advances in Practice Medicine-Rheumatology
CiteScore
3.60
自引率
3.20%
发文量
197
审稿时长
11 weeks
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