SAPHO综合征患者临床特点分析

Clinical Characteristics of SAPHO Syndrome

目的探讨SAPHO综合征的临床表现、诊断、治疗。方法对9例SAPHO综合征患者的临床表现、影像、治疗资料进行回顾性分析。结果9例患者中,8例有典型皮肤掌跖脓疱病,6例有胸锁关节炎;9例均行全身骨显像检查,提示受累骨关节放射性浓聚影,其中1例发现有“牛头征”;1例进行胸锁关节病理组织检查,提示活检部位炎细胞浸润,炎性肉芽组织形成。患者均给予非甾体抗炎药、免疫抑制剂、云克治疗,1例患者加用TNF-α拮抗剂英夫利昔单抗治疗6个月。结论SAPHO综合征的诊断主要基于2012年Nguyen等提出的SAPHO综合征诊断标准,全身骨显像检查对于诊断有重要参考价值。非甾体抗炎药联合免疫抑制剂是主要治疗方法,生物制剂可用于常规治疗效果不佳者。该病临床罕见,临床中需要警惕该病。

Objective To investigate the clinical manifestations,diagnosis and treatment of SAPHO syndrome.Methods The clinical manifestations,imaging and treatment data of 9 patients with SAPHO syndrome were retrospectively analyzed.Results Among the 9 patients,8 had typical skin palmoplantar pustular disease and 6 had sternoclavicular arthritis.All 9 cases underwent whole-body bone imaging examination,which indicated the radioactive concentrated shadow of the affected bone and joint,and 1 case was found to have"bullhead sign".The pathological examination of sternoclavicular joint in 1 case showed infiltration of inflammatory cells and formation of inflammatory granulation tissue at the biopsy site.All patients were treated with non-steroidal antiinflammatory drugs,immunosuppressants and 99Tc-MDP,and 1 patient was treated with Infliximab,a TNF-αantagonist,for 6 months.Conclusion The diagnosis of SAPHO syndrome is mainly based on the diagnostic criteria of SAPHO syndrome proposed by Nguyen et al in 2012,and whole-body bone imaging has important reference value for diagnosis.Non-steroidal anti-inflammatory drugs combined with immunosuppressive agents are the main treatment methods,and biological agents can be used for patients who do not respond to conventional treatment.This disease is rare in clinic and should be vigilant in clinic.