From the Department of Rheumatology, University Hospitals of Leicester NHS Trust, Leicester Royal Infirmary, Leicester, United Kingdom.
Joint Department of Medical Imaging, University Health Network, Toronto, Canada.
J Clin Rheumatol. 2020 Apr;26(3):99-103. doi: 10.1097/RHU.0000000000000959.
The coexistence of joint hypermobility syndrome (JHS) and spondyloarthropathy (SpA) presents a challenging clinical conundrum due to the contradictory clinical signs that may be present. Classic features such as restricted spinal movement or early morning back stiffness may not be present. Timely diagnosis and appropriate management of these patients are difficult as they tend to have lower scores on validated objective measures.
We performed a medical records review study to identify patients with both JHS and SpA who had presented to the Leicester Spondyloarthropathy clinic. Patients were diagnosed with axial SpA if they met the Assessment of SpondyloArthritis international Society classification criteria. Their imaging was reviewed by a consultant musculoskeletal radiologist.
Four cases were identified from the patient database (female; average age, 37.5 years). All patients presented with lower back pain or sacroiliac joint pain but preserved spinal movement with a negative Schober's test. Two had a history of symptoms for more than 10 years. All had a Beighton score of greater than 6. Three of the patients were HLA positive, and 3 had a positive family history. All patients thus far have had their symptoms adequately controlled on nonsteroidal anti-inflammatory drugs and physiotherapy.
The coexistence of JHS and SpA is rare but important to recognize. These patients are difficult to diagnose as they may present late because of preserved spinal movements. It is unclear whether the preserved flexibility masks the true extent of disease or whether clinically they represent a less severe disease phenotype.
联合活动过度综合征 (JHS) 和脊柱关节炎 (SpA) 的共存存在一个具有挑战性的临床难题，因为可能存在相互矛盾的临床体征。受限的脊柱运动或早期晨僵等经典特征可能不存在。由于这些患者的验证性客观指标得分往往较低，因此及时诊断和适当管理这些患者较为困难。
我们进行了一项病历回顾研究，以确定在莱斯特脊柱关节炎诊所就诊的同时患有 JHS 和 SpA 的患者。如果患者符合评估脊柱关节炎国际协会分类标准，则被诊断为轴向 SpA。由一位顾问肌肉骨骼放射科医生对其影像学进行评估。
从患者数据库中确定了 4 例（女性；平均年龄 37.5 岁）。所有患者均出现下腰痛或骶髂关节疼痛，但脊柱运动正常，Schober 试验阴性。其中 2 例有超过 10 年的症状史。所有患者的 Beighton 评分均大于 6。3 例患者 HLA 阳性，3 例有阳性家族史。迄今为止，所有患者的症状均通过非甾体抗炎药和物理治疗得到了充分控制。
JHS 和 SpA 的共存虽然罕见，但需要认识到这一点。由于脊柱运动保持正常，这些患者可能会出现迟诊，因此诊断较为困难。尚不清楚保持的灵活性是否掩盖了疾病的真实程度，或者临床上它们是否代表了一种较不严重的疾病表型。
