SAPHO综合征164例临床表型聚类分析的临床意义  

Cluster analysis of clinical phenotypes and its implication in 164 patients with Synovitis-Acne-Pustulosis-Hyperostosis-Osteitis syndrome

作　　者：曹逸涵 徐平 李忱[2] 刘晋河[2] 郝伟欣[2] 张文[3] 董振华[2] Cao Yihan;Xu Ping;Li Chen;Liu Jinhe;Hao Weixin;Zhang Wen;Dong Zhenhua

机构地区：[1]中国医学科学院北京协和医院放射科,100730 [2]中国医学科学院北京协和医院中医科,100730 [3]中国医学科学院北京协和医院风湿免疫科,100730 [4]北京协和医学院临床医学系

出　　处：《中华风湿病学杂志》2018年第12期793-797,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).

摘　　要：目的采用聚类分析方法探讨SAPHO综合征的临床表型及其意义。方法招募2004—2015年在北京协和医院就诊的164例满足Kahn和Khan提出的SAPHO综合征诊断标准的患者,收集其人口统计学、临床表现、实验室检查及影像学数据。纳入其中11项最能反映SAPHO综合征患者特征的变量,采用迭代分区的K-means算法进行聚类分析,最佳的类别数量通过肘部法则、Silhouettes系数法并结合临床意义确定。结果聚类分析示最佳类别数目为2类。类别1(116例)以中轴骨关节受累为特征,发病年龄[(38±11)岁]较高,重度痤疮比例较低(11.2%);类别2(41例)无中轴骨关节受累,发病年龄较低[(33±8)岁;U=1 800,P=0.010],重度痤疮比例较高(26.8%;χ^24.567,P=0.033)。类别1较类别2患者在基线前接受了更为积极的治疗,其TNF-α拮抗剂的使用率高于类别2(分别为32.8%,2.4%;χ^21 672.5,P<0.01),双膦酸盐的使用率亦显著高于类别2(分别为39.7%,19.5%;χ^21 962,P=0.032)。然而,在基线时,类别1的疾病活动度评分高于类别2[BASDAI分别为(3.5±1.8),(2.8±2.0);U=1 800,P=0.010] [BASFI分别为(2.4±2.3),(1.5±1.7);U=1 791,P=0.009],炎症指标亦高于类别1[ESR分别为(34.9±2.9)mm/1 h,(19.0±14.6)mm/1 h;U=1 204.5,P<0.01][hs-CRP分别为(16±19)mg/L,(8±11)mg/L;U=1 628,P=0.01]。结论以是否存在中轴骨关节受累为主要特征,SAPHO综合征中存在2种疾病表型,其在发病年龄、重度痤疮发生率及疾病严重程度等方面差异均有统计学意义。ObjectiveTo use cluster analysis to explore the clinical phenotypes of Synovitis-Acne-Pustulosis-Hyperostosis-Osteitis (SAPHO) syndrome.MethodsOne hundred and sixty-four patients fulfilled the Kahn and Khan's criteria for SAPHO syndrome were recruited in Peking Union Medical College Hospital from 2004 to 2015. For all patients, demographics, clinical, laboratory and imaging data were collected. Cluster analysis was performed using an iterative partitioning K-means method including 11 variables that was most characteristic in patients with SAPHO syndrome. The optimal number of clusters was determined by the elbow method and Silhouettes coefficient in combination with clinical significance.Results An optimal of two phenotypes with distinct clinical features were identified. Cluster 1 was characterized by axial skeletal involvement with older age at onset [(38±11) years] and lower prevalence of severe acne (11.2%);Cluster 2 had no axial involvement with younger age at onset [(33±8) years;U=1 800, P=0.010] and higher prevalence of severe acne (26.8%;χ^24.567, P=0.033). Cluster 1 patients had been treated more aggressively by baseline compared with Cluster 2 patients;and were more frequently prescribed TNF-α inhibitors (32.8% vs 2.4%;χ^21 672.5, P<0.01) and bisphosphonates (39.7% vs 19.5%;χ^21962, P=0.032). Nonetheless, the disease activity indices were significantly higher at baseline in Cluster 1 than Cluster 2 patients [Bath ankylosing spondylitis disease activity index (BASDAI) (3.5±1.8) vs (2.8±2.0);U=1 800, P=0.010] [Bath ankylosing spondylitis functional Index(BASDFI) (2.4±2.3) vs (1.5±1.7);U=1 791, P=0.009). Moreover, Cluster 1 patients had significantly increased inflammatory markers at baseline compared with Cluster 2 patients [erythrocyte sedi-ment-ation rate(ESR) (34.9±2.9) mm/1 h vs (19.0±14.6) mm/1 h;U=1 204.5, P<0.01] [high-sensitivity C-reactive protein (hs-CRP) (16±19) mg/L vs (8±11) mg/L;U=1 628, P=0.01].Conclusion Char-acterized by the presence or absence of axial skeletal involvement,

关 键 词：SAPHO综合征 聚类分析 脊柱 骶髂关节 发病年龄 

分 类 号：R597[医药卫生—内科学]