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滑膜炎、痤疮、脓疱病、骨肥厚和骨炎综合征:164例患者队列的单中心研究

Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome: a single centre study of a cohort of 164 patients.

作者信息

Li Chen, Zuo Yuzhi, Wu Nan, Li Li, Li Feng, Zhang Weihong, Xu Wenrui, Zhao Xue, Jing Hongli, Pan Qingqing, Zhou Weixun, Shi Xiaohua, Fan Yu, Wang Jianyi, Liu Sen, Liu Zhenlei, Zhang Fengchun, Zeng Xiaofeng, Chen Hui, Zhang Siya, Liu Jinhe, Qiu Guixing, Wu Zhihong, Dong Zhenhua, Zhang Wen

机构信息

Department of Traditional Chinese Medicine.

Department of Orthopedic Surgery.

出版信息

Rheumatology (Oxford). 2016 Jun;55(6):1023-30. doi: 10.1093/rheumatology/kew015. Epub 2016 Feb 25.

Abstract

OBJECTIVE

The aim was to assess the clinical, laboratory and radiological features of SAPHO syndrome.

METHODS

We recruited all patients presenting to Peking Union Medical College Hospital from 2004 to 2015 diagnosed with SAPHO syndrome. The medical data, laboratory test results and imaging were collected for all patients.

RESULTS

One hundred and sixty-four patients (111 women and 53 men) were recruited to our cohort. The mean age of the patients was 40.71 years. Nine patients had osteoarticular symptoms without skin involvement. One hundred and forty-three and 25 patients had palmoplantar pustulosis and severe acne, respectively. Psoriasis vulgaris was accompanied by palmoplantar pustulosis or severe acne in 24 patients. One hundred and sixty-four patients suffered from pain in the anterior chest wall, followed by spine (12 in the cervical region, 36 in the thoracic region and 111 in the lumbosacral region) and peripheral joint (136 patients) involvement. None of the patients had IBD. The hs-CRP level was increased in 70.8% patients. Only 2.4% were HLA-B27 positive. CT scan indicated osteolysis, sclerosis and hyperostosis in the anterior chest wall and spine in SAPHO syndrome patients. The bull-horn sign was the typical characteristic of SAPHO syndrome seen in bone scintigraphy images. One hundred and thirty-one (79.9%), 85 (51.8%), 100 (61%) and 54 (32.9%) patients took NSAIDs, CSs, DMARDs and oral bisphosphonates, respectively.

CONCLUSION

SAPHO syndrome is predominant in middle-age women, characterized by dermatological and osteoarticular manifestations with unknown aetiology. CT scan and bone scintigraphy are useful for diagnosis. There is still no standard treatment to control the disease.

摘要

目的

评估滑膜炎、痤疮、脓疱病、骨肥厚和骨髓炎综合征(SAPHO综合征)的临床、实验室及影像学特征。

方法

我们纳入了2004年至2015年在北京协和医院就诊且被诊断为SAPHO综合征的所有患者。收集了所有患者的医疗数据、实验室检查结果及影像学资料。

结果

我们的队列纳入了164例患者(111例女性和53例男性)。患者的平均年龄为40.71岁。9例患者有骨关节症状但无皮肤受累。143例和25例患者分别有掌跖脓疱病和重度痤疮。24例寻常型银屑病患者伴有掌跖脓疱病或重度痤疮。164例患者均有前胸壁疼痛,其次为脊柱(颈椎12例、胸椎36例、腰骶椎111例)和外周关节(136例患者)受累。所有患者均无炎症性肠病。70.8%的患者高敏C反应蛋白水平升高。仅2.4%的患者HLA - B27呈阳性。CT扫描显示SAPHO综合征患者前胸壁和脊柱有骨质溶解、硬化及骨质增生。骨闪烁显像图像中“牛角征”是SAPHO综合征的典型特征。分别有131例(79.9%)、85例(51.8%)、100例(61%)和54例(32.9%)患者服用了非甾体抗炎药(NSAIDs)、糖皮质激素(CSs)、改善病情抗风湿药(DMARDs)和口服双膦酸盐。

结论

SAPHO综合征在中年女性中占主导,以病因不明的皮肤和骨关节表现为特征。CT扫描和骨闪烁显像有助于诊断。目前仍没有控制该病的标准治疗方法。

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