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SAPHO综合征13例临床特征、疗效观察及文献复习 被引量:2

Clinical characteristics and therapeutic effect of SAPHO syndrome: report of 13 cases and literature review
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摘要 目的总结SAPHO综合征(主要包括滑膜炎、痤疮、脓疱病、骨肥厚和骨炎、以骨关节及皮肤受累为主要表现的风湿性疾病)的临床特征,为临床避免临床漏诊、误诊及探讨有效治疗方法提供经验。方法收集2012年1月至2016年12月包头医学院第一附属医院住院及门诊诊治的SAPHO综合征13例,分析其临床表现、实验室检查及治疗转归,并结合文献复习进行讨论。结果 13例中男性3例,女性10例。病程中骨关节受累部位:上胸壁处13例,外周关节11例,骶髂关节7例,脊柱关节6例。外周关节受累的主要部位依次为:肩关节、手小关节、膝关节、肘关节。13例中12例行^(99m)Tc标记的亚甲基二磷酸盐(^(99m)Tc-MDP)全身骨扫描,1例行正电子发射计算机体层摄影(PET-CT)检查,结果均提示:受累关节部位骨盐代谢增强。9例患者均同时给予非甾体抗炎药、甲氨蝶呤及阿仑膦酸钠联合治疗,症状明显缓解,但皮疹仍有反复;2例采用肿瘤坏死因子(TNF)-α拮抗剂英夫利昔单抗注射液联合甲氨蝶呤片治疗效果好;1例英夫利昔单抗治疗失败的难治性患者应用阿达木单抗有效。结论 SAPHO综合征皮肤改变主要为掌跖脓疱病;骨关节改变以上胸壁处受累最多见。全身骨扫描和PET-CT检查对该病的诊断最有价值。非甾体抗炎药联合甲氨蝶呤及阿仑膦酸钠对改善关节症状效果明显。TNF-α拮抗剂对难治性SAPHO综合征有效;重症患者治疗效果不好者,可尝试阿达木单抗。 Objective To summarize the clinical features of synovitis-acne-pustulosis-hyperostosis-osteomyelitis(SAPHO) syndrome (a rheumatic diseases characterized by main clinical manifestations of osteoarticular and skin involvements including synovitis, acne, pustulosis, hyperostosis and osteitis) to provide experience for avoiding clinical missed diagnosis, misdiagnosis and exploring effective treatment methods. Methods Thirteen patients with SAPHO syndrome diagnosed and treated in inpatient and outpatient of the First Affiliated Hospital of Baotou Medical College, from January 2012 to December 2016 were selected, and the clinical manifestation, laboratory examination and treatment outcome were analyzed. Results There were 3 males and 10 females in 13 cases. In the course of the disease, the sites of bone and joint involvement were upper chest wall ( 13 cases), peripheral joints ( 11 cases ), sacroiliac joints ( 7 cases ), spinal joints ( 6 cases ). The sites of peripheral joints involvement mainly were shoulder joint, hand small joint, knee joint, elbow joint in turn. In 13 cases ,99~nTc labeled methylene diphosphonate (99mTc-MDP) whole body bone scan was performed in 12 cases, and positron emission tomography (PET-CT) was performed in one case, and all resuhs prompted that bone salt metabolism increased in the affected joint site. Nine cases were treated with combined thearpy [ nonsteroidal anti - inflammatory drugs ( NSAIDs ) , methotrexate and alendronate ] , and their symptoms were markedly relieved, but the skin rash was still repeated. Two cases were treated with tumor necrosis factor (TNF)-ct antagonist infliximab injection combined with methotrexate tablet, and the effect was good. One refractory patients was treated with adalimumab thearpy effectively after failed to infliximab treatment. Conclusions Palmoplantar pustulosis is the main skin change in SAPHO syndrome. Upper chest wall involvement is the most common osteoarticular change. The whole body bone scan and PET-CT examination is the most valuable method to diagnosis of the disease. NSAIDs combined with methotrexate and alendronate has obvious effect on improving joint symptoms. TNF-α antagonist is effective for refractory SAPHO syndrome, and the severe patients with poor efficacy can try adalimumab treatment.
出处 《中国临床研究》 CAS 2017年第7期974-979,共6页 Chinese Journal of Clinical Research
关键词 SAPHO综合征 掌跖脓疱病 骨关节病变 99mTc标记的亚甲基二磷酸盐全身骨扫描 正电子发射计算机体层摄影 非甾体抗炎药 免疫抑制剂 双膦酸盐 肿瘤坏死因子-Α拮抗剂 SAPHO syndrome Palmoplantar pustulosis Osteoarticular lesion 99m Tc labeled methylene diphosphonate whole body bone scan Positron emission tomography Nonsteroidal anti-inflammatory drugs Immunosuppressor Diphosphonate Tumor necrosis factor-α antagonist
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