Three patterns of osteoarticular involvement in SAPHO syndrome: a cluster analysis based on whole body bone scintigraphy of 157 patients

Three patterns of osteoarticular involvement in SAPHO syndrome: a cluster analysis based on whole body bone scintigraphy of 157 patients
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DOI:
10.1093/rheumatology/key415
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发表时间:
2019-06-01
期刊:
影响因子:
5.5
通讯作者:
Zhang, Wen
Zhang, Wen
中科院分区:
医学1区
文献类型:
--
作者:
Cao, Yihan;Li, Chen;Zhang, Wen

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目标.探讨SAPHO综合征的骨关节受累模式。收集164例SAPHO综合征患者中157例患者的基线临床特征和99 m-MDP全身骨显像(WBBS)资料。采用Ward最小方差法对12个最常受累的骨关节部位进行系统聚类分析。骨关节受累有三种不同的类型:脊柱型(70例患者,44.6%),主要为胸椎、腰椎或骶椎病变;肋型前肋病变52例(33.1%),尤其是第一肋;胸锁型(35例,22.3%),以胸骨和双侧胸锁病变为主,以典型的牛头征为特征。值得注意的是,共有77例(49%)患者在WBBS上显示肋骨病变,其中61.3%累及第一根肋骨。有趣的是,脊柱型患者在皮肤表现的发病年龄比胸锁型(P = 0.036)和肋型(P = 0.035)。胸锁型病程明显长于肋型(P = 0.001)和脊柱型(P < 0.001)。SAPHO综合征的骨关节受累可分为三种不同的类型,具有不同的临床特征。SAPHO综合征的肋骨受累,以前认识不足,可能定义了该疾病的一个独特亚型。
Objectives. To explore the patterns of osteoarticular involvement in SAPHO syndrome.Methods. Baseline clinical characteristics and imaging data of Tc-99m-MDP whole-body bone scintigraphy (WBBS) were collected from 157 out of 164 patients diagnosed with SAPHO syndrome. The twelve most frequently involved osteoarticular sites were analysed by hierarchical cluster analysis with the Ward minimum-variance method.Results. Three distinctive patterns of osteoarticular involvement were identified: the spinal type (70 patients, 44.6%), with predominantly thoracic, lumbar or sacral vertebral lesions; the costal type (52 patients, 33.1%), with lesions of anterior ribs, particularly the first ribs; and the sternoclavicular type (35 patients, 22.3%), with predominantly sternal and bilateral sternoclavicular lesions, characterized by the typical bullhead sign. Notably, a total of 77 (49%) patients exhibited lesions of ribs on WBBS, of which 61.3% involved the first ribs. Interestingly, patients of spinal type were older at onset of cutaneous manifestations than those of sternoclavicular type (P = 0.036) and costal type (P = 0.035). The disease course was remarkably longer in sternoclavicular type than costal type (P = 0.001) and spinal type (P < 0.001).Conclusion. The osteoarticular involvement in SAPHO syndrome can be categorized as three distinct patterns with different corresponding clinical features. The costal involvement in SAPHO syndrome, which was under-recognized previously, may define a distinct sub-type of the disease.