SAPHO综合征164例首发症状临床分析  

Clinical analysis of the initial symptoms among 164 synovitis, acne, pustulosis, hyperostosis, osteitis syndrome patients

作　　者：赵冰彬 吴遐[2] 李忱[1] 曹选涵 刘晋河[1] 张文[3] 董振华[1] 郝伟欣[1] Zhao Bingbin;Wu Xia;Li Chen;Cao Yihan;Liu Jinhe;Zhang Wen;Dong Zhenhua;Hao Weixin(Department of Traditional Chinese Medicine, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China;Department of Internal Medicine, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, Chin)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院中医科,100730 [2]中国医学科学院北京协和医学院北京协和医院内科,100730 [3]中国医学科学院北京协和医学院北京协和医院风湿免疫科,100730

出　　处：《中华风湿病学杂志》2018年第5期298-302,共5页Chinese Journal of Rheumatology

基　　金：国家重点研发计划罕见病临床队列研究（2016YFC0901501）;首都卫生发展科研专项（2016-4-40112）;中国医学科学院医学与健康科技创新工程项目（2017-12M-3-001）

摘　　要：目的探究SAPHO综合征患者的首发症状，并分析其临床及实验室特征。方法回顾性分析2004—2015年期间就诊于北京协和医院的164例SAPHO综合征患者的首发症状，并收集其人口统计学、临床表现、实验室检查及影像学数据。计量资料采用独立样本t检验和Mann-Whitney U检验，计数资料采用χ2检验，分析不同首发症状SAPHO综合征患者的临床特征差异。结果84例（51.2%）SAPHO综合征患者的首发症状为皮肤损害，29例（17.7%）骨关节表现先于皮损出现，余42例（25.6%）皮损与骨关节症状同时出现。133例（81.1%）患者皮肤和骨关节损害的症状出现时间间隔在2年内，但间隔最长可达35年。此外，以骨关节表现为首发症状的患者发病年龄高于以皮损为首发症状的患者[（41±10）岁和（36±11）岁，t=-2.174，P=0.032]；HLA-B27的阳性率在以骨关节表现为首发症状的患者高于以皮损为首发症状的患者（10%和0，P=0.016）。尽管接受了更高强度的治疗，但以骨关节表现为首发症状的患者基线时hs-CRP水平仍较高[5.42（1.88，12.70）和11.60（3.76，22.08）mg/L，Z=-2.096，P=0.036]。结论多数SAPHO综合征患者的首发表现为皮肤损害，而以骨关节损害为首发表现的患者比例随着发病年龄增长而增加。ObjectiveTo explore the initial symptoms of synovitis, acne, pustulosis, hyperostosis, osteitis （SAPHO） syndrome, and to analyze the clinical and laboratory characteristics of these patients. MethodsWe retrospectively analyzed the initial symptoms of 164 patients diagnosed with SAPHO syndrome presented at Peking Union Medical College Hospital from 2004 to 2015, and their clinical, laboratory, and radiological data were collected. The t test, Mann-Whitney U test and chi-square test were used to compare the clinical differences between the SAPHO patients with different initial symptoms. ResultsAmong the 164 patients recruited, 84（51.2%） had skin lesions before osteoarticular symptoms, whereas 29（17.7%） after and42（25.6%） simultaneously. Nine （5.5%） patients had no skin lesions. The time interval between onset of skin and osteoarticular lesions was less than 2 years in 133（81.1%） patients, but up to 35 years at most. Interestingly, a significantly higher age at onset was observed in patients with osteoarticular symptoms prior to skin lesions than those after [（41±10） years vs （36±11） years, t=-2.174, P=0.032]. Moreover, positive HLA-B27 was more frequently detected in patients having osteoarticular symptoms as the initial presentations （10.3% vs 0, P=0.016）. Although treated more aggressively before baseline, patients presented with osteoarticular symptoms prior to skin lesions had significantly higher level of hs-CRP at baseline compared with those after [5.42（1.88, 12.70） mg/L vs 11.60（3.76, 22.08） mg/L, Z=-2.096, P=0.036].ConclusionSkin lesions tend to appear prior to osteoarticular symptoms in most SAPHO syndrome patients. The percentage of patients who developed skin lesions after osteoarticular symptoms increase with age at onset.

关 键 词：获得性骨肥大综合征 体征和症状 皮肤表现 

分 类 号：R597[医药卫生—内科学]