摘要
目的 SAPHO综合征属罕见病,由于对其临床特点认识不足,往往造成漏诊和误诊,总结北京协和医院12例SA-PHO综合征患者,旨在提高对本病的早期诊断,探讨本病的治疗方案。方法对12例SAPHO综合征患者的临床资料、治疗转归进行总结分析。结果 12例患者中男性2例,女性10例。皮损表现为掌跖脓疱病10例,暴发性痤疮者l例,无皮疹者1例。骨关节受累上胸壁处受累12例,外周关节受累10例,骶髂关节受累8例,脊柱关节受累3例。11例行99mTc-MDP全身骨扫描,均不同程度提示上胸壁处异常放射浓聚。12例患者均给予非甾体抗炎药(NSAID),糖皮质激素和(或)改变病情抗风湿药(DMARD),其中8例加用抗肿瘤坏死因子-α拮抗剂,4例在病程中配合中药治疗。随访过程中未见严重骨骼关节并发症。结论本组资料以女性多见,皮肤改变以掌跖脓疱病多见,骨关节改变以上胸壁处受累多见,99mTc-MDP全身骨扫描对本病的敏感性及特异性均较高。单用NSAID类药物,症状多难控制;糖皮质激素和改变病情抗风湿药可部分改善症状;抗肿瘤坏死因子-α可显著改善症状,但停药易复发;中医药治疗本病有一定作用,有望成为治疗本病的新方法。
Objective SAPHO syndrome is a very rare disease. It is difficult to make this diagnosis correctly without well - known clinical features of SAPHO syndrome. We collected 12 cases diagnosed SAPHO syndrome in Peking Union Medical College Hospital to summarize the characters of SAPHO, for early diagnosis and better treatment of this disease. Methods Clinical data of 12 cases of syno- vitis,acne,pustulosis,hyperostosis,osteitis （SAPHO） syndrome were analysed. Results There were 2 males and 10 females among the 12 patients. Dermatological manifestations included palmoplantar pustulosis in 10 patients and severe ache in 1 patient. Osteoarticular manifes- tations included anterior chest wall involved in 12 patients, peripheral joints involved in 10 patients, sacroiliac joints involved in 8 pa- tients, and vertebral joints involved in 3 patients. Eleven patients had performed 99mTc - MDP whole body bone scintigraphy （WBBS） ex- amination, and abnormal metabolic foci at anterior chest wall were found in each case. All 12 patients were treated with non - steroidal an- ti- inflammatory drugs （NSAIDs） and disease modifying anti -rheumatic drugs （DMARDs）. Eiqht patients were treated with anti - tumor necrosis factor - α （TNF - α） inhibitors, and 4 patients were treated with herbal medicine as further addition therapy. Conclusion Female patients were dominant in our series of cases. Palmoplantar pustulosis was the most common manifestation of skin. Among the os- teoarticular manifestations, anterior chest wall had the most popular involvement. 99mTc - MDP WBBS examination has relatively high sen- sitivity and specificity towards the diagnosis of SAPHO syndrome. Treatment with NSAIDs alone was difficult to relieve the symptoms. Gly- cocorticosteroids with（or） DMARDs could partially ameliorate the disease. Anti -TNF-or inhibitors could improve the symptoms appar- ently, but cessation of the drugs could induce the relapse. Traditional chinese medication was demonstrated as a good new method of SA- PHO syndrome treatment.
出处
《医学研究杂志》
2012年第12期117-122,共6页
Journal of Medical Research
关键词
SAPHO综合征
临床表现
治疗
Synovitis ache pustulosis hyperostosis osteitis （SAPHO） syndrome
Clinical manifestation
Treatment
