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关节过度活动与埃勒斯-当洛斯综合征

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Bendy joints that can slip or ache — the spectrum from benign hypermobility to hEDS, and why strengthening is the mainstay.

拇指向近端屈曲至前臂方向，提示关节活动度异常增大。
关节过度活动性的典型体征——拇指可向近端（前臂方向）过度屈曲。 Dagger9977 / Wikimedia Commons, CC BY-SA 3.0

本页面由机器翻译，尚未经临床医生审核。英文版本为权威版本。

您的感受

您可能一直是那个“关节过度灵活”的人，能够将拇指向后弯曲至前臂，肘关节过度伸展，或者把自己折叠成让旁人看了都皱眉的姿势。对许多人来说，这种灵活性只是一种无害的特征，完全不会带来麻烦。

然而，对一些人来说，这需要付出代价。活动范围超出正常限度的关节也可能出现疼痛，尤其是在活动后或一天结束时。您可能会注意到某些关节会发出咔哒声、钝响或感觉松动，仿佛要脱位，有时确实会发生脱位。在上肢中，肩部、小指关节和拇指基底是常见的“罪魁祸首”：肩部部分滑出（半脱位）或完全脱位，手指向错误方向弯曲，以及拇指关节在捏握时失去支撑。损伤的恢复时间可能比您预期的要长，且同一个关节可能反复出现问题。

关节过度活动常伴随其他特征。有些人皮肤柔软或富有弹性，且容易淤青。许多人描述疲劳感与其活动量不成比例，站立时头晕或心跳加速，以及腹胀或反流等消化症状。并非每个人都有所有这些症状，但如果您的关节松弛与其中几项症状相符，它们之间可能存在关联。

实际发生了什么

您的关节由结缔组织（韧带、肌腱以及包裹每个关节的关节囊）连接和支撑，而这种组织的一个关键构成成分是名为胶原蛋白的蛋白质。在关节活动度过大的人群中，胶原蛋白和结缔组织通常比常人更具弹性和延展性。支撑结构会给予更多的缓冲，因此关节的活动范围可以超出正常限度。

这属于一个谱系。一端是简单的关节活动度过大，通常不会引起任何问题。当松弛的关节开始产生疼痛、不稳定或反复受伤时，医生称之为关节活动度过大谱系障碍。另一端是高活动性埃勒斯-当洛斯综合征（hEDS），此时更具延展性的结缔组织表现更为显著，且更可能伴随更广泛的全身性特征（皮肤、瘀伤、疲劳、肠道症状以及头晕/自主神经症状）。这些类别之间的界限并不清晰，且个体在谱系中的位置可能随时间推移而改变。为了帮助描绘这一情况，医生通常会结合您的完整病史，使用一套简单的弯曲测试，称为Beighton评分。

这对您的关节之所以重要，归结于一个核心理念：如果被动支撑结构（韧带和关节囊）对关节的固定作用较弱，关节就必须更多地依赖周围肌肉来维持稳定。当这些肌肉强壮且协调良好时，关节保持稳定。当它们疲劳或未针对该功能进行充分训练时，关节会发生滑移、酸痛，并在多年后可能比正常情况下磨损得更快。

肩部有一种值得了解的特定模式。单次损伤可能导致正常肩部向单一方向脱位，而松弛的肩部往往同时感到多个方向的不稳定，向前、向后或向下滑动。医生将此称为多方向不稳定。这也意味着这些肩部倾向于更频繁地发生部分滑移并自我复位（半脱位），而非完全脱位。这一点至关重要，因为这种类型的不稳定对建立肌肉控制反应最佳，而对单独手术的反应最差。

我们可以采取的措施

最重要的信息是：关节过度活动症（hypermobility）和超移动性结缔组织病（hEDS）无法治愈，但非常可控，而管理的基石并非药物或手术，而是锻炼稳定关节的肌肉。

物理治疗和针对性强化训练是主要手段。了解关节过度活动症的物理治疗师会与您合作，针对您最困扰的关节建立控制力和力量。对于上肢，这通常意味着锻炼肩胛骨和肩袖肌肉，以及控制拇指和手指的小肌肉。目标是训练您的肌肉承担韧带功能减弱后所需的稳定工作。这是一个渐进且持续的过程，而非速效方案，但对大多数人而言，这正是改变现状的关键。

围绕这一核心，还有几项辅助措施：

  • 节奏调整与关节保护： 将活动分散到全天，避免使松弛关节承受压力的极限位置，并调整执行费力或重复性任务的方式。
  • 支具与贴扎： 选择性使用，以在建立力量或度过急性发作期时支撑关节，而非作为永久性的拐杖。
  • 疼痛管理： 采用简单策略，必要时使用药物，并关注睡眠、疲劳及其他全身性症状，因为这些都会影响关节的感觉。

手术需谨慎对待。 对于上肢外科医生而言，这是一个真正重要的问题。当关节（最常见的是肩关节）在良好的康复后仍反复脱位时，可考虑进行稳定手术。但是，最初导致关节松弛的同一类延展性组织，也会使手术修复的可靠性降低，且愈合方式可能不同，因此标准稳定手术在长期内出现拉伸或失败的风险更高。鉴于此，手术仅保留给经过仔细筛选的问题，规划时须充分考虑患者的关节过度活动情况，并且术前术后必须配合强化训练方案。康复是保护手术效果的关键。

预期情况

关节过度活动是身体结构的一部分，因此不会消失，但通过正确的管理，其影响可以发生巨大变化。许多坚持强化训练计划的人发现，他们的关节变得更加稳定，疼痛得到缓解，关节脱位和半脱位的频率显著降低。进展通常是渐进而非突发的，以月为单位衡量，并且通过长期保持肌肉的良好状态（而非在情况好转后停止训练），才能最好地维持这些获益。

将这一过程视为与身体的长期合作关系，而非一次性解决的问题，会有所帮助。急性发作仍可能发生，尤其是在疾病后、生长突增期或活动量减少的时期之后，您将更善于识别早期迹象并加以预防。如果存在更广泛的症状（如疲劳、头晕、肠道症状），在关注关节的同时兼顾这些症状，通常会使整体情况更易于管理。在合理的计划下，大多数人能够保持活跃，并从事对他们重要的事情。

何时就医

  • 关节反复脱位或部分滑出，尤其是肩关节，或首次脱位后无法复位。
  • 致残性疼痛，或尽管休息并采取常规措施，疼痛仍持续加重。
  • 影响日常生活的关节不稳： 因关节反复失效，导致抓握、提举、工作或睡眠困难。
  • 新发或迅速恶化的关节问题，或手臂或手部出现麻木、刺痛或无力。
  • 如果您怀疑患有高活动性谱系障碍或hEDS并希望得到专业评估（特别是当关节松弛伴随皮肤问题、瘀伤、疲劳、头晕或肠道症状时），值得寻求审慎的评估，以便制定合适的计划。

深入探讨

Advanced reading: the deeper science (optional)

本节内容超出了您自身治疗决策所需的范围。关节过度活动值得额外阅读，因为“自信的故事”——即关节松弛是胶原蛋白基因问题，且松弛的关节可以简单地通过手术收紧——在证据面前，这两点均无法成立。

最常见的类型无已知遗传病因

2017年国际分类承认13种埃勒斯-当洛综合征（Ehlers-Danlos syndrome）。其中十二种由20个已鉴定基因中的变异引起，对于这十二种类型，确诊需要分子层面的确认 [1]。

第十三种是高活动型EDS（hypermobile EDS），其患病率远高于其他类型，且其分子基础仍未知 [1]。目前没有针对该类型的基因检测。它是唯一一种纯粹基于临床标准进行诊断的类型，这也是为何与那些以实验室结果告终的疾病相比，该诊断可能让人感觉令人沮丧地主观。

这一点对于如何解释该疾病至关重要。对于经典型和血管型EDS，说高活动性“归因于胶原蛋白基因的遗传差异”是准确的，因为其致病变异是已知的。但对于高活动型EDS，这只是一个合理的假设，而非已证实的事实。诚实的立场是，我们可以可靠地描述组织行为，但尚无法指明其分子病因。

Beighton评分的可靠性高于其声誉

九分制Beighton评分常被视为粗糙且依赖检查者。这一批评缺乏依据。一项纳入1,333名参与者的系统综述发现，该评分具有良好至优秀的评分者间和评分者内信度，且这一结论在不同背景和经验的评分者中均成立 [2]。

因此，该测量方法是可靠的。真正的局限性在于：该评分采样小指、拇指、肘部、膝部和脊柱，良好的评分反映的是全身性松弛度，它从未被设计用于评估眼前单个肩关节的稳定性。

上肢关节过度活动性实际预测的内容

这是肩关节门诊中至关重要的数据。一项针对 2,335 名运动员的系统评价和荟萃分析发现，关节过度活动性与 肩关节损伤风险增加三倍 相关 [3]。作者对基础证据的质量持适当的谨慎态度，但其方向和幅度不容忽视。

该风险并不局限于上肢。全身性关节松弛与前交叉韧带重建术后 移植物失败率增加和疗效较差 相关 [4]，同一组织在不同关节中表现出相同的行为。

手术效果较差，且数据具体

这是文献中最令人不安的部分，也是最有用的部分。

一项针对 1,769 名患者的系统综述和荟萃分析发现，接受全关节置换术的埃勒斯-当洛综合征（Ehlers-Danlos syndrome）患者，其 全因翻修、不稳定、无菌性松动和伤口并发症 的风险显著增加 [5]。另一项关于埃勒斯-当洛综合征各亚型骨科手术结果的独立综述也得出了相同结论：与一般人群相比，手术效果较差，且并发症发生频率更高 [6]。

上述内容并不意味着手术是错误的。它表明，与一般人群引用的数据相比，预期的获益较小，预期的并发症发生率较高，因此手术指征的阈值应相应调整。如果您具有关节过度活动性，已发表的手术成功率大多并非在类似您这样的人群中测得的。

对于不稳定肩关节，康复并非安慰奖

多向不稳定是指肩关节在多个方向上松弛，而非从单一方向脱位，这是典型的肩关节过度活动问题，也是上述观点最清晰的例证。

当康复确实失败后需要手术时，关节镜下关节囊折叠术与开放关节囊移位术在复发性不稳定、重返运动及并发症方面的发生率相当 [7][8]，且关节镜入路引起的术后僵硬较少 [9]。一种历史技术——热缩关节囊术——效果不佳，以至于荟萃分析建议避免使用该技术 [9]。

顺序本身就是信息。所有这些综述都将手术定位为康复失败后的后续步骤，而非其替代方案，因为收紧关节囊并不能改变其构成组织，而肌肉仍然是系统中唯一可以被强化的部分。

参考文献

[1] Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):8-26. https://doi.org/10.1002/ajmg.c.31552

[2] Bockhorn LN, Vera AM, Dong D, Delgado DA, Varner KE, Harris JD. Interrater and intrarater reliability of the Beighton score: a systematic review. Orthop J Sports Med. 2021;9(1). https://doi.org/10.1177/2325967120968099

[3] Liaghat B, Pedersen JR, Young JJ, Thorlund JB, Juul-Kristensen B, Juhl CB. Joint hypermobility in athletes is associated with shoulder injuries: a systematic review and meta-analysis. BMC Musculoskelet Disord. 2021;22(1). https://doi.org/10.1186/s12891-021-04249-x

[4] Krebs NM, Barber-Westin S, Noyes FR. Generalized joint laxity is associated with increased failure rates of primary anterior cruciate ligament reconstructions: a systematic review. Arthroscopy. 2021;37(7):2337-47. https://doi.org/10.1016/j.arthro.2021.02.021

[5] Subramanian T, Uzzo RN, Lama J, Mazzucco M, Ortiz S, Gausden EB. Outcomes of total joint arthroplasty in patients who have Ehlers-Danlos syndrome: a systematic review and meta-analysis. J Arthroplasty. 2025;40(8):2186-95.e1. https://doi.org/10.1016/j.arth.2025.01.018

[6] Yonko EA, LoTurco HM, Carter EM, Raggio CL. Orthopedic considerations and surgical outcomes in Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2021;187(4):458-65. https://doi.org/10.1002/ajmg.c.31958

[7] Longo UG, Rizzello G, Loppini M, Locher J, Buchmann S, Maffulli N, et al. Multidirectional instability of the shoulder: a systematic review. Arthroscopy. 2015;31(12):2431-43. https://doi.org/10.1016/j.arthro.2015.06.006

[8] Jacobson ME, Riggenbach M, Wooldridge AN, Bishop JY. Open capsular shift and arthroscopic capsular plication for treatment of multidirectional instability. Arthroscopy. 2012;28(7):1010-17. https://doi.org/10.1016/j.arthro.2011.12.006

[9] Chen D, Goldberg J, Herald J, Critchley I, Barmare A. Effects of surgical management on multidirectional instability of the shoulder: a meta-analysis. Knee Surg Sports Traumatol Arthrosc. 2015;24(2):630-9. https://doi.org/10.1007/s00167-015-3901-4

Reviewed by Dr Kieran Hirpara · 24 June 2026.

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c. indicate the Licensed Material is licensed under this Public License, and include the text of, or the URI or hyperlink to, this Public License.

2. You may satisfy the conditions in Section 3(a)(1) in any reasonable manner based on the medium, means, and context in which You Share the Licensed Material. For example, it may be reasonable to satisfy the conditions by providing a URI or hyperlink to a resource that includes the required information.

3. If requested by the Licensor, You must remove any of the information required by Section 3(a)(1)(A) to the extent reasonably practicable.

4. If You Share Adapted Material You produce, the Adapter's License You apply must not prevent recipients of the Adapted Material from complying with this Public License.

Section 4 -- Sui Generis Database Rights.

Where the Licensed Rights include Sui Generis Database Rights that apply to Your use of the Licensed Material:

a. for the avoidance of doubt, Section 2(a)(1) grants You the right to extract, reuse, reproduce, and Share all or a substantial portion of the contents of the database for NonCommercial purposes only;

b. if You include all or a substantial portion of the database contents in a database in which You have Sui Generis Database Rights, then the database in which You have Sui Generis Database Rights (but not its individual contents) is Adapted Material; and

c. You must comply with the conditions in Section 3(a) if You Share all or a substantial portion of the contents of the database.

For the avoidance of doubt, this Section 4 supplements and does not replace Your obligations under this Public License where the Licensed Rights include other Copyright and Similar Rights.

Section 5 -- Disclaimer of Warranties and Limitation of Liability.

a. UNLESS OTHERWISE SEPARATELY UNDERTAKEN BY THE LICENSOR, TO THE EXTENT POSSIBLE, THE LICENSOR OFFERS THE LICENSED MATERIAL AS-IS AND AS-AVAILABLE, AND MAKES NO REPRESENTATIONS OR WARRANTIES OF ANY KIND CONCERNING THE LICENSED MATERIAL, WHETHER EXPRESS, IMPLIED, STATUTORY, OR OTHER. THIS INCLUDES, WITHOUT LIMITATION, WARRANTIES OF TITLE, MERCHANTABILITY, FITNESS FOR A PARTICULAR PURPOSE, NON-INFRINGEMENT, ABSENCE OF LATENT OR OTHER DEFECTS, ACCURACY, OR THE PRESENCE OR ABSENCE OF ERRORS, WHETHER OR NOT KNOWN OR DISCOVERABLE. WHERE DISCLAIMERS OF WARRANTIES ARE NOT ALLOWED IN FULL OR IN PART, THIS DISCLAIMER MAY NOT APPLY TO YOU.

b. TO THE EXTENT POSSIBLE, IN NO EVENT WILL THE LICENSOR BE LIABLE TO YOU ON ANY LEGAL THEORY (INCLUDING, WITHOUT LIMITATION, NEGLIGENCE) OR OTHERWISE FOR ANY DIRECT, SPECIAL, INDIRECT, INCIDENTAL, CONSEQUENTIAL, PUNITIVE, EXEMPLARY, OR OTHER LOSSES, COSTS, EXPENSES, OR DAMAGES ARISING OUT OF THIS PUBLIC LICENSE OR USE OF THE LICENSED MATERIAL, EVEN IF THE LICENSOR HAS BEEN ADVISED OF THE POSSIBILITY OF SUCH LOSSES, COSTS, EXPENSES, OR DAMAGES. WHERE A LIMITATION OF LIABILITY IS NOT ALLOWED IN FULL OR IN PART, THIS LIMITATION MAY NOT APPLY TO YOU.

c. The disclaimer of warranties and limitation of liability provided above shall be interpreted in a manner that, to the extent possible, most closely approximates an absolute disclaimer and waiver of all liability.

Section 6 -- Term and Termination.

a. This Public License applies for the term of the Copyright and Similar Rights licensed here. However, if You fail to comply with this Public License, then Your rights under this Public License terminate automatically.

b. Where Your right to use the Licensed Material has terminated under Section 6(a), it reinstates:

1. automatically as of the date the violation is cured, provided it is cured within 30 days of Your discovery of the violation; or

2. upon express reinstatement by the Licensor.

For the avoidance of doubt, this Section 6(b) does not affect any right the Licensor may have to seek remedies for Your violations of this Public License.

c. For the avoidance of doubt, the Licensor may also offer the Licensed Material under separate terms or conditions or stop distributing the Licensed Material at any time; however, doing so will not terminate this Public License.

d. Sections 1, 5, 6, 7, and 8 survive termination of this Public License.

Section 7 -- Other Terms and Conditions.

a. The Licensor shall not be bound by any additional or different terms or conditions communicated by You unless expressly agreed.

b. Any arrangements, understandings, or agreements regarding the Licensed Material not stated herein are separate from and independent of the terms and conditions of this Public License.

Section 8 -- Interpretation.

a. For the avoidance of doubt, this Public License does not, and shall not be interpreted to, reduce, limit, restrict, or impose conditions on any use of the Licensed Material that could lawfully be made without permission under this Public License.

b. To the extent possible, if any provision of this Public License is deemed unenforceable, it shall be automatically reformed to the minimum extent necessary to make it enforceable. If the provision cannot be reformed, it shall be severed from this Public License without affecting the enforceability of the remaining terms and conditions.

c. No term or condition of this Public License will be waived and no failure to comply consented to unless expressly agreed to by the Licensor.

d. Nothing in this Public License constitutes or may be interpreted as a limitation upon, or waiver of, any privileges and immunities that apply to the Licensor or You, including from the legal processes of any jurisdiction or authority.

Creative Commons is not a party to its public licenses. Notwithstanding, Creative Commons may elect to apply one of its public licenses to material it publishes and in those instances will be considered the “Licensor.” The text of the Creative Commons public licenses is dedicated to the public domain under the CC0 Public Domain Dedication. Except for the limited purpose of indicating that material is shared under a Creative Commons public license or as otherwise permitted by the Creative Commons policies published at creativecommons.org/policies, Creative Commons does not authorize the use of the trademark "Creative Commons" or any other trademark or logo of Creative Commons without its prior written consent including, without limitation, in connection with any unauthorized modifications to any of its public licenses or any other arrangements, understandings, or agreements concerning use of licensed material. For the avoidance of doubt, this paragraph does not form part of the public licenses.

Creative Commons may be contacted at creativecommons.org.