SAPHO综合征5例临床分析

Clinical analysis of 5 cases with SAPHO syndrome

关键词:  SAPHO 综合征  临床表现  影像学  诊断

Key words:  [Key words]　SAPHO syndrome  clinical manifestation  imaging  diagnosis

摘要:

[摘要]　目的　了解SAPHO综合征的临床特征，尽早对该病做出诊断。　方法　收集5例SAPHO综合征患者的血常规(routine blood tes)，肝肾功，血沉，C反应蛋白及免疫球蛋白，补体，HLA-B27等相关化验检查及胸锁关节、骶髂关节及腰椎CT，全身骨扫描检查临床资料。5例均有不同程度的皮肤、骨改变，其中2例有骶髂关节受累，1例耻骨联合受累，1例脊柱受累，3例有外周关节受累。4例HLA-B27阴性，1例阳性。5例患者分别予非甾体抗炎药，糖皮质激素及免疫抑制剂，肿瘤坏死因子拮抗剂，中药治疗。　结果　1例应用类克治疗，症状很快得到改善。3例分别在激素，NSAIDS基础上联合雷公藤，甲氨蝶呤，柳氮磺吡啶治疗，1例应用中药治疗，临床上关节肿痛好转。　结论　本病好发于中年女性，以掌跖脓疱病及上胸壁、胸锁关节受累多见，若无典型皮肤改变，容易误诊，影像学检查是必要的。早期进行治疗可以有效减轻症状，干预疾病进展。

Abstract:

[Abstract] Objective To investigate the clinical characteristics of SAPHO syndrome and to improve the recognition of the disease. Methods The 5 patients of SAPHO syndrome were tested for routine peripheral blood, liver function, renal function, erythrocyte sedimentation rate (ESR), c reactive protein (CRP), immunoglobulin, complement and human leucocyte antiget-B27 (HLA-B27) et al, and received sternoclavicular joint CT, sacroiliac joint CT, lumbar vertebra bone scan. The patients were given non-steroidal anti-inflammatory drug (NSAID), glucocorticoids, tumor necrosis factor-α (TNF-α) and chinese medicine. Results Clinical manifestations included skin, joints and bones. Sacroiliac joints were involved in 2 patients, including pubic symphysis and spine in 1 patient each. Peripheral joints were involved in 3 patients. One patient was positive for HLA-B27; whereas the other four were negative. One patient received etanercept; 3 patients were given NSAIDS and glycocorticosteroid plus methotrexate, sulfalazine, or Glucosidorum Tripterygll Totorum; one patient was given chinese medicine. All of the treatments had a good effect. Conclusion Females are dominant in our cases. Palmoplantar pustulosis and anterior chest wall, sternoclavicular joint are the most common manifestations．It is easy to be misdiagnosed if the patient does not have typical skin symptoms. Early treatment can reduce clinical symptoms and slow the progression.