中轴骨受累的SAPHO综合征患者临床及影像学分析

The clinical and imaging manifestations of SAPHO syndrome involvement in axial bones

目的:探讨SAPHO综合征累及中轴骨患者临床及影像学表现,提高对此类疾病的认知率及诊断准确率。方法:回顾性分析42例(男16例,女26例)中轴骨受累SAPHO综合征患者的临床资料及影像学资料,总结临床特点、病变范围、部位、数目及骨质改变情况。结果:中轴骨受累患者主要临床表现为骨及关节症状(42例)及皮损表现(40例),26例有中轴骨症状(颈部、背部、腰骶部及下颌骨疼痛)。中轴骨累及包括脊柱(28例),骶髂关节(18例),颅面骨(5例);脊柱中胸椎受累比例(23例)及胸椎个数(47个)最多,23例表现为多椎体受累(病灶数≥2)。特征性影像征象:前胸壁骨显像“牛头征”,CT相邻椎体“接吻征”(5例),MRI椎体角呈圆弧形异常信号(6例),骶髂关节以单侧受累为主(12例),表现为骶髂关节炎性病变,部分骨质硬化,关节腔间隙存在;颅面骨以单侧下颌骨受累为主(4例),表现为骨质破坏改变。结论:SAPHO综合征累及中轴骨不少见,临床表现及影像学表现异质性较大,多模态影像学诊断可以提高对该疾病诊断效能。

Objective:To investigate the clinical and imaging manifestations of SAPHO syndrome patients with axial skeletal involvement and to improve the recognition rate and diagnostic accuracy.Methods:The clinical data and imaging data of 42 patients(16 males and 26 females)with SAPHO syndrome involving the axial bones were retrospectively analyzed,and the clinical characteristics,lesion range,location,number,and bone changes were summarized.Results:The main clinical manifestations of patients with axial bone involvement were bone and joint symptoms(42 cases)and skin lesions(40 cases),with 26 cases having axial bone symptoms(pain in the neck,back,lumbosacral region,and mandible).The axial bone involvement included the spine(28 cases),sacroiliac joints(18 cases),and craniofacial bones(5 cases).The proportion of thoracic vertebrae involvement(23 cases)and the number of thoracic vertebrae(47)were the highest,with 23 cases showing multiple vertebral involvement(lesion number≥2).Characteristic imaging signs included the"bull-head sign"on the anterior chest wall on ECT,the"kissing sign"on CT adjacent vertebral bodies(5 cases),the abnormal signal of the round arc shape on MRI vertebral body angle(6 cases),and the unilateral involvement of the sacroiliac joint(12 cases),which manifested as inflammatory lesions of sacroilitis,partial bone sclerosis,and presence of joint space.The craniofacial bones were mainly affected by unilateral mandibular involvement(4 cases),manifested as bone destruction changes.Conclusion:SAPHO syndrome involving the axial bones is not uncommon,and the clinical and imaging manifestations are highly heterogeneous.Multimodal imaging diagnosis can improve the diagnostic efficiency of this disease.