摘要目的 通过分析滑膜炎-痤疮-脓疱病-骨肥厚-骨炎(SAPHO)综合征患者的临床特征及影像学表现,提高对该综合征的认识.方法 对2014年1月至2017年1月的14例(男8例、女6例,年龄18～61岁)SAPHO患者的临床资料进行回顾性分析.收集所有患者的临床表现、实验室检查[红细胞沉降率(ESR)、C反应蛋白(CRP)、C3补体、C4补体、人类白细胞抗原(HLA)-B27]、病理及影像学(CT、骨显像)资料.结果 SAPHO综合征患者的主要特征是皮肤损害和骨关节损害,其中皮肤损害主要表现为脓疱、脱屑及红斑;骨关节损害主要表现为胸锁关节骨肥厚、僵硬性脊柱骨肥厚、外周关节炎、骶髂关节炎.14例SAPHO患者中,ESR加快5例,CRP异常5例,C3补体异常3例,C4补体异常1例,HLA-B27均阴性.全身骨显像中,14例患者均出现异常放射性浓聚影,主要累及前胸壁,脊柱、骨盆和长骨次之.患者胸部CT影像学表现为关节骨质破坏、肥厚融合以及关节间隙变窄,其中对称性病变占多数.病理学检查中,9例表现为炎性反应,并不存在特异性.结论 SAPHO综合征是以皮肤、骨以及关节异常为主要特征的疾病.全身骨显像可以早期发现全身骨损害,CT能够及时发现骨质变化以及关节软组织病变.

abstractsObjective To improve the recognition of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome by investigating its clinical features and imaging manifestations. Methods The data of 14 patients with SAPHO syndrome (8 males, 6 females, age range: 18-61 years) between January 2014 and January 2017 were retrospectively analyzed. The clinical features, the results of laboratory (erythrocyte sedimentation rate (ESR), C reaction protein (CRP), complement component 3 (C3) and component 4 (C4), human leukocyte antigen (HLA)-B27) tests, pathology and imaging examinations (CT and whole-body bone im-aging) were collected. Results SAPHO syndrome was mainly characterized by skin damage and bone joint damage. The main manifestations of skin lesions were pustules, desquamation and erythema. Those of bone and joint injury were sternocostoclavicular hyperostosis, stiff spinal hyperostosis, peripheral arthritis, and sacroiliitis. There were 5 with fasting ESR, 5 with abnormal CRP, 3 with abnormal C3, and 1 with abnormal C4 among 14 patients. HLA-B27 was negative in all patients. Whole-body bone imaging revealed that 14 pa-tients had abnormal radioactivity, mainly involving the anterior chest wall, spine, pelvis and long bone. Chest CT manifestations of the patients were the destruction of joint bone, the fusion of the hypertrophy and the narrowing of the joint space. Symmetric lesions accounted for the majority. Pathology examination showed non-specific inflammation in 9 patients. Conclusions SAPHO syndrome is a disease characterized by skin, bone and joint abnormalities. Whole-body bone imaging can find systemic bone damage in early stage, and CT can detect bone changes and soft tissue lesions.