SAPHO综合征的临床表现与治疗

Diagnosis and preliminary treatment results of SAPHO syndrome

目的分析滑膜炎、痤疮、脓疱病、骨肥厚和骨炎(synovitis,acne,pustulosis,hyperostosis,osteitis,SAPHO)综合征的临床表现并总结治疗结果。方法回顾性分析本中心2002年1月至2018年1月,以骨病变首诊的SAPHO综合征15例。根据病史、出现滑膜炎、痤疮和脓疱病的临床体征、检查判断骨肥厚和骨炎的影像学表现、血液生化检验以及穿刺活检病理学检查进行本病的诊断。通过随访比较疼痛评分(visual analogue scale,VAS)和化验指标的改善情况评价治疗结果。结果本组SAPHO综合征男6例,女9例,平均年龄和中位年龄分别为36.5和35.0(8~68)岁,平均病史和中位病史时间分别为37.9和12.0(2~120)个月,平均随访时间和中位随访时间分别为37.3和21.0(4~187)个月。以胸锁关节疼痛就诊6例,长骨疼痛就诊9例。其中股骨7例,胫骨2例,10例为骨单发病灶,5例为多发骨病灶。10例为同期伴有皮肤损害,5例不同时期伴有皮肤损害。12例接受了穿刺活检,病理均表现为慢性炎性细胞浸润、反应性新生骨。采用非甾体消炎镇痛药物(non-steroidal and anti-inflammatory drugs,NSAIDs)、双磷酸盐和抗生素进行治疗,在此基础上其中1例接受了手术治疗。治疗前后VAS疼痛评分比较,显著降低(Z=-3.447,P=0.001),治疗前后中性粒细胞比例变化(Z=-1.255,P=0.205)、治疗前后血沉比较(erythrocyte sedimentation rate,ESR)(Z=-3.044,P=0.002)、治疗前后C反应蛋白比较(C-reactive protein,CRP)(Z=-2.934,P=0.003)均显著降低。结论 SAPHO综合征发病率低,容易误诊和漏诊。非甾体消炎镇痛结合双磷酸盐和抗生素药物可以明显缓解疼痛,缓解炎性指标,初期治疗随访结果满意。

Objective To demonstrate the clinical manifestations of synovitis, acne, pustulosis, hyperostosis and osteomyelitis（ SAPHO） syndrome and summarize the preliminary treatment results. Methods A retrospective analysis of 15 cases chief complaint with bone disease of SAPHO syndrome from Jan 2002 to Jan 2018 was conducted. Diagnosis was made based on the history, clinical signs of synovitis, acne and pustulosis, imaging features of hyperostosis and osteomyelitis, hematologic examination and histopathology. The initial Visual Analogue Scale（ VAS） and hematologic test were applied in comparison with the results after treatment for the improvement evaluation. Results There were 6 males and 9 females in this series of SAPHO syndrome. The average and median age was 36.5 and 35.0 years（ range： 8-68 years） respectively. The mean and median relevant medical history of SAPHO was 37.9 and 12.0 months（ range： 2-120 months） respectively. The mean and median follow-up time was 37.3 and 21.0 months（ range： 4-187 months） respectively. Six patients had chief complaint with sternoclavicular joint pain, 9 patients with extremity long bone trouble（ 7 cases in the femur and 2 in the tibia）. Ten cases were of single lesion and 5 of multiple lesions. Ten cases had synchronous skin lesion and 5 cases had heterochronia skin lesion. Biopsy was performed in 12 patients, showing chronic inflammatory cell infiltration and reactive new bone formation. Nonsteroidal and anti-inflammatory drugs（ NSAIDs）, bisphosphonates and antibiotics were used to relieve symptoms. One patient underwent surgical treatment. The VAS was significantly reduced after treatment（ Z =-3.447, P = 0.001）. The proportion of neutrophils had no changes after treatment（ Z =-1.255, P = 0.205）, the Erythrocyte Sedimentation Rate（ ESR）（ Z =-3.044, P = 0.002） and C-reactive protein（ CRP） were also significantly decreased after treatment（ Z =-2.934, P = 0.003）. Conclusions The morbidity of SAPHO syndrome is very low, but it has high misdiagnosis rate. NSAIDs and bisphosphonates combined with antibiotic drug can significantly relieve the pain and inflammatory biomarkers. The preliminary treatment results are satisfactory in the follow-up.