Chinese General Practice ›› 2022, Vol. 25 ›› Issue (21): 2617-2623.DOI: 10.12114/j.issn.1007-9572.2022.0097

• Original Research • Previous Articles     Next Articles

Characteristics of SAPHO Syndrome: Clinical Analysis of 19 Cases

  

  1. Xiyuan Hospital of China Academy of Chinese Medical Sciences, Beijing100091, China
  • Received:2022-01-15 Revised:2022-03-11 Published:2022-07-20 Online:2022-05-24
  • Contact: Qiuai KOU
  • About author:
    SUN W T, KOU Q A. Characteristics of SAPHO syndrome: clinical analysis of 19 cases[J]. Chinese General Practice, 2022, 25 (21) : 2617-2623.

SAPHO综合征19例临床特征分析

  

  1. 100091 北京市,中国中医科学院西苑医院
  • 通讯作者: 寇秋爱
  • 作者简介:
    孙文婷,寇秋爱. SAPHO综合征19例临床特征分析[J].中国全科医学,2022,25(21):2617-2623. [www.chinagp.net] 作者贡献:孙文婷进行文章的构思与设计,文章的可行性分析及文章的数据收集、整理,撰写论文;孙文婷、寇秋爱进行论文的修订,英文的修订;寇秋爱负责文章的质量控制及校审,对文章整体负责,监督管理。

Abstract:

Background

SAPHO syndrome is a rare chronic inflammatory disease of unknown causes with various clinical manifestations, which involves bone joint and skin tissue, and has no specific diagnostic indicators and uniform treatment strategy.

Objective

To analyze the clinical characteristics of 19 cases of SAPHO syndrome, increasing clinicians' recognition of the disease.

Methods

A retrospective analysis was performed on clinical characteristics 19 cases of SAPHO syndrome recruited from Rheumatology Clinic, Xiyuan Hospital of China Academy of Chinese Medical Sciences from October 2013 to December 2021, including the first symptoms, bone joint and skin involvement, laboratory and imaging findings, comorbidities prevalence, surgical history, medication, follow-up, and misdiagnosis prevalence.

Results

There were 17 female cases and two male cases, with an average age of (43±12) years and an average duration of 1 year. The first symptom was bone joint pain (n=14) or dermatological manifestations (n=5) . Sixteen patients (84.2%) had bone joint and skin involvement, and the other three (15.8%) had only bone joint involvement. Anterior chest wall was the most commonly involved site, followed by the spine, peripheral joints, and sacroiliac joints. Palmoplantar pustulosis was the most common manifestation of skin involvement (16 cases, 84.2%) , and among these cases, two also had psoriasis-like lesions on the lower extremities and seven also had damaged fingernails of both hands. Eighteen cases had whole-body bone scintigraphy, and were detected with an average number of sites of bone joint involvement of (3±1) . Misdiagnosis occurred in 10 cases (52.6%) , and the average diagnostic delay for them was (3±5) years. Palmoplantar pustulosis was found in all 6 cases (31.6%) with tonsillitis, and the symptoms were improved with no recurrence within one year in two out of three cases undergoing tonsillectomy.

Conclusion

As a rare disease mainly involving bone joint and the skin, SAPHO syndrome has a high misdiagnosis rate. The recognition of the common involved parts of the disease and whole-body bone scintigraphy and other imaging examinations are helpful to make a proper diagnosis. Tonsillitis may be related to the development of the disease.

Key words: Acquired hyperostosis syndrome, SAPHO syndrome, Disease attributes

摘要:

背景

SAPHO综合征是一种罕见的影响骨关节和皮肤组织的慢性炎症性疾病,该病病因及发病机制不明,临床表现多样,目前尚无特异性的诊断指标及统一的治疗策略。

目的

分析19例SAPHO综合征患者的临床特征,以提高临床医生对该病的认识。

方法

回顾性分析2013年10月至2021年12月在中国中医科学院西苑医院风湿科门诊就诊的19例SAPHO综合征患者的临床特征,分析患者的首发症状特点、骨关节及皮肤受累特点、实验室及影像学检查、合并症及手术史、用药及随访情况、误诊情况。

结果

19例患者中女17例,男2例;平均年龄为(43±12)岁,中位病程为1年。首发症状均为骨关节疼痛或皮肤病表现,以骨关节疼痛为首发症状的患者14例,以皮肤病为首发症状5例;骨关节及皮肤均受累患者16例(84.2%),仅骨关节受累、无皮肤受累的患者3例(15.8%)。骨关节受累最常见的部位是前胸壁,其次是脊柱、外周关节、骶髂关节。皮肤受累最常见的表现是掌跖脓疱疮(16例,84.2%),其中2例伴下肢银屑病样皮损,7例伴双手指甲损坏。18例行全身骨扫描检查,平均骨关节受累位点数为(3±1)个。10例(52.6%)患者曾经历误诊,平均延迟诊断时间为(3±5)年。6例(31.6%)合并扁桃体炎的患者中,均存在掌跖脓疱疮,3例患者行扁桃体切除术,2例术后症状好转、1年内无复发。

结论

SAPHO综合征是一种主要累及骨关节及皮肤的罕见病,临床误诊率较高,对该病常见受累部位的认识及全身骨显像等影像学检查有助于明确诊断,扁桃体炎可能与该病的发生、发展有一定关系。

关键词: 获得性骨肥大综合征, SAPHO综合征, 疾病特征