可弯曲型埃勒斯-当洛综合征与高活动型谱系障碍之间自我报告症状和合并症的异同
Similarities and differences in self-reported symptoms and comorbidities between hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders.
作者信息
Darakjian Ashley A, Bhutani Mira, Fairweather DeLisa, Kocsis S Christian, Fliess Jessica J, Khatib Sami, Weigel Gabe J, McCabe Elizabeth J, Balamurugan Varsini, Perona Evan E, Gehin Jessica M, Whelan Emily R, Jain Angita, Sledge Hanna, Hodge David O, Rozen Todd D, Farraye Francis A, Soyer Ozan, Cheung Joseph, Grach Stephanie L, Shirey David, Gajarawala Shilpa, Munipalli Bala, Shufelt Chrisandra L, Knight Dacre R T, Bruno Katelyn A
机构信息
Department of Cardiovascular Medicine, Mayo Clinic, Jacksonville, FL, USA.
Center for Clinical and Translational Science, Mayo Clinic, Rochester, MN, USA.
出版信息
Rheumatol Adv Pract. 2024 Nov 4;8(4):rkae134. doi: 10.1093/rap/rkae134. eCollection 2024.
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 ( = 1389), 20.3% with hEDS ( = 423), 10.6% with historic HSD (H-HSD) or localized HSD (L-HSD) ( = 256) (hypermobile controls) and 2.6% were not diagnosed with hEDS, HSD, H-HSD or L-HSD ( = 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.
目的
患有可弯曲性埃勒斯-当洛综合征（hEDS）和可弯曲性谱系障碍（HSD）的患者会经历各种各样的症状和系统紊乱。本研究旨在确定被诊断为hEDS或HSD的患者在115种自我报告的症状和合并症方面是否存在差异。
方法
在本研究中，我们分析了来自EDS诊所 intake问卷的自我报告数据，这些数据来自被诊断为hEDS、HSD或无可弯曲性疾病的患者。
结果
从2019年11月1日至2024年3月7日，EDS诊所接待了2088名患者。根据2017年诊断标准，66.5%被诊断为HSD（n = 1389），20.3%被诊断为hEDS（n = 423），10.6%被诊断为既往HSD（H-HSD）或局限性HSD（L-HSD）（n = 256）（可弯曲性对照），2.6%未被诊断为hEDS、HSD、H-HSD或L-HSD（n = 55）（对照）。在hEDS和HSD中高患病率出现的症状/合并症包括关节疼痛（hEDS 82.0%，HSD 88.9%）、过敏（hEDS 77.0%，HSD 77.0%）、半脱位（hEDS 71.2%，HSD 72.6%）、脑雾（hEDS 70.0%，HSD 74.7%）、头痛（hEDS 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%）的症状/合并症在hEDS患者中自我报告的频率明显更高，但在HSD患者中为42/115（37%）。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、胃肠道、睡眠和心理症状/合并症。
结论
尽管我们发现hEDS/HSD患者自我报告的一些症状和合并症存在重叠，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这两种诊断之间存在关键差异，这表明hEDS和HSD可能是不同的病症。
Zotero 插件