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作 者:王志芳 章振林[1] WANG Zhi-fang;ZHANG Zhen-lin(Shanghai Clinical Research Center of Bone Disease,Department of Osteoporosis and Bone Disease,Shanghai Jiao Tong University Affiliated Sixth People s Hospital,Shanghai 200233,China;Department of Endocrinology,The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)
机构地区:[1]上海交通大学附属第六人民医院骨质疏松与骨病专科,上海市骨疾病临床研究中心,上海200233 [2]郑州大学第一附属医院内分泌科,郑州450052
出 处:《中华骨质疏松和骨矿盐疾病杂志》2022年第4期397-402,共6页Chinese Journal Of Osteoporosis And Bone Mineral Research
基 金:上海市申康医院发展中心新兴前沿技术联合攻关项目(SHDC2018120)。
摘 要:分析3例滑膜炎-痤疮-脓疱病-骨肥厚-骨炎(synovitis-acne-pustulosis-hyperostosis-osteitis,SAPHO)综合征患者的临床资料,包括临床表现、实验室检查及影像学资料,同时结合文献进行讨论。本文3例患者男性1例,女性2例,起病年龄46~56岁,病程0.5~8年,均同时存在骨关节和皮肤损害,骨关节损害出现均早于皮肤损害,均主要累及胸肋骨和胸锁关节。实验室相关指标检查均在正常范围[血沉(erythrocyte sedimentation rate,ESR),C反应蛋白(C-reactive protein,CRP),抗核抗体,人类白细胞抗原B27(human leukocyte antigen B27,HLA-B27),碱性磷蛋白(alkaline phosphatase,ALP)、Ⅰ型胶原羧基端肽交联(carboxy-telopeptide of typeⅠcollagen,β-CTX),骨钙素等]。全身骨显像均可见典型“牛头征”,局部SPECT/CT断层显像均可见病变部位骨质硬化、关节受累,其中2例可见溶骨性破坏。总之,SAPHO综合征是一种以皮肤、骨及关节异常为特征的罕见疾病,临床表现多样,易误诊。当骨痛时有溶骨性、硬化性或肥厚性骨病变等影像学表现时,应考虑该病的可能,而全身骨显像有助于早期诊断。The clinical data of 3 cases with synovitis-acne-pustulosis-hyperostosis-osteitis(SAPHO)syndrome were analyzed,including clinical presentations,laboratory examination and imaging examinations,and the related literatures were reviewed.There were 1 male and 2 females.The onset age was between 46 to 56 years with the course of disease of 0.5-8 years.All patients had bone and skin lesions,bone and joint damage appeared earlier than skin damage,and thoracic ribs and sternoclavicular joints were mainly involved.All relevant indicators of laboratory examination were normal.Typical“bull s head”can be seen in bone scintigraphy.The local SPECT/CT tomography showed that all patients had osteosclerosis,joints involvement,and 2 patients had osteolysis.SAPHO syndrome is a rare disease characterized by abnormal skin,bone and joint.The clinical manifestations were varied and easy to be misdiagnosed.In the presence of osteolytic,sclerosing,or hypertrophic bone lesions and pain,the possibility of the disease should be considered.The whole-body bone scintigraphy is helpful for early diagnosis.
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