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中国全科医学 ›› 2019, Vol. 22 ›› Issue (36): 4482-4487.DOI: 10.12114/j.issn.1007-9572.2019.00.364

• 专题研究 • 上一篇    下一篇

SAPHO综合征患者临床特征分析

王卫涛1，2，郑朝晖1，2，谢荣华1，2，吴振彪1，2*   

  1. 1.710000陕西省西安市，空军军医大学第一附属医院临床免疫科　2.710000陕西省西安市，全军风湿病研究所
    *通信作者：吴振彪，教授，主任医师，博士生导师；E-maill：wuzhenbiao@fmmu.edu.cn
  • 出版日期:2019-12-20 发布日期:2019-12-20

Clinical Features of SAPHO Syndrome　

WANG Weitao1，2，ZHENG Zhaohui1，2，XIE Ronghua 1，2，WU Zhenbiao1，2*   

  1. 1.Department of Clinical Immunology，the First Affiliated Hospital of Air Force Medical University，Xi'an 710000，China
    2.People's Liberation Army Specialized Research Institute of Rheumatism & Immunity，Xi'an 710000，China
    *Corresponding author：WU Zhenbiao，Professor，Chief physician，Doctoral supervisor；E-maill：wuzhenbiao@fmmu.edu.cn
  • Published:2019-12-20 Online:2019-12-20

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摘要： 背景　SAPHO综合征作为临床少见病之一，近年来对其认知不断深入，但多样化的临床表现及规范的诊疗措施仍需不断总结、摸索及进一步研究。目的　分析总结SAPHO 综合征的临床特征、诊断与治疗。方法　回顾性选择2012年2月—2018年5月空军军医大学（原第四军医大学）西京医院收治的12例SAPHO综合征患者，分析其发病特点、临床表现、影像学表现、血清学检查、皮肤组织活检、治疗及随访。结果　12例患者中男8例、女4例；发病年龄为17~53岁，平均（37.8±11.8）岁；病程为2个月~25年。12例患者均有皮肤及骨与关节病变，其中掌跖脓疱疹、胸锁关节肿痛、肩锁关节疼痛为主要临床表现，全身骨扫描为重要检查手段，典型“牛头征”有重要诊断价值。血清学检查无特异性，急性期患者可有红细胞沉降率、C反应蛋白升高，皮肤组织活检可见典型“脓疱疹”改变。目前治疗仍以经验性治疗为主，免疫抑制剂联合非甾体抗炎药治疗效果良好，多数患者在规律药物治疗基础上病情控制较平稳。结论　SAPHO综合征男女均可发病，早期症状不典型，首发症状多为皮肤或关节病变，全身骨扫描“牛头征”为该病特征性表现，而皮肤组织活检可见炎症性改变，对于疾病诊断及鉴别具有重要价值。该病在早诊断、早治疗的情况下多数预后良好。

关键词: 获得性骨肥大综合征, 脓疱病, 掌跖, 关节炎, 体征和症状

Abstract: Background　The cognition of the SAPHO syndrome，a rare disease，has made continuous progress in recent years.However，standardized diagnostic and therapeutic regimens for the disease need to be further explored，studied and summarized due to diversified manifestations.Objective　To analyze and summarize the clinical features，diagnosis and treatment of SAPHO syndrome.Methods　Twelve cases of SAPHO syndrome were retrospectively selected from the First Affiliated Hospital of Air Force Medical University during February 2012 to May 2018.Pathogenic characteristics，clinical features，imaging manifestations，serological makers，skin biopsy，treatment and follow-up results were analyzed.Results　In this group，there were 8 males and 4 females，with an average age of onset of （37.8±11.8） years （range 17-53），and the course of the disease ranged from 2 months to 25 years.All cases presented some lesions of the skin，bone and joint to varying degrees，with palmoplantar impetigo，sternoclavicular joint swelling and pain，and acromioclavicular joint pain as the main clinical manifestations.Whole body bone scintigraphy is an important detective approach，and the detection of bull's head sign，a typical sign of SAPHO syndrome on bone scintigraphy，is of great diagnostic significance.Serological examination showed no pathognomonic changes，but in the acute phase，elevated erythrocyte sedimentation rate and C-reactive protein levels were found.Typical pustules could be found in skin biopsy.Experiential therapies are the major treatments at present.Immunosuppressive agents combined with non-steroidal drugs can achieve good effectiveness.The conditions of most cases with regular pharmacological treatment were controlled and became stable.Conclusion　SAPHO syndrome can occur in both men and women.The early symptoms are not typical，and the initial symptoms are mostly skin or joint lesions.Bull's head sign on bone scintigraphy，a pathognomonic sign of SAPHO syndrome，in combination with inflammatory changes detected by skin biopsy，are important indicators for the diagnosis and differential diagnosis of this disease.Most patients with early diagnosis and treatment have a good prognosis.

Key words: Acquired hyperostosis syndrome, Impetigo, Palms and soles, Arthritis, Signs and symptoms

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