SAPHO综合征14例临床特征与治疗  

Clinical Characteristics and Treatment of 14 Patients with SAPHO Syndrome

作　　者：石静艺 迪丽努尔·阿不都热依木 赵娟[2] 康晓静[2] SHI Jingyi;Dilinuer Abudureyimu;ZHAO Juan;KANG Xiaojing(Graduate School of Xinjiang Medical University,Urumqi 830001,China;Department of Dermatology and Venereology,People's Hospital of Xinjiang Uygur Autonomous Region,Xinjiang Clinical medical Research Center For Dermatologic Diseases,Xinjiang Key Laboratory of Dermatology Research(XJYS1707),Urumqi 830001,China)

机构地区：[1]新疆医科大学研究生学院,新疆乌鲁木齐830001 [2]新疆维吾尔自治区人民医院皮肤性病科新疆皮肤病临床医学研究中心新疆皮肤病研究重点实验室(XJYS1707),新疆乌鲁木齐830001

出　　处：《中国皮肤性病学杂志》2023年第2期168-173,共6页The Chinese Journal of Dermatovenereology

基　　金：新疆维吾尔自治区重点研发计划项目(2021B03001)。

摘　　要：目的探讨SAPHO综合征患者的临床特征。方法对14例SAPHO综合征患者的临床资料、治疗方案及临床疗效进行回顾性分析。结果本组14例患者中男8例,女6例,平均发病年龄(28.43±13.38)岁。14例均有皮肤损害(掌跖脓疱病8例,寻常型痤疮2例,囊肿型痤疮3例,聚合型痤疮1例)。影像学中病变累及的骨关节依次为胸前壁(13/14)、骶髂关节(11/14)、四肢骨关节(6/14)、脊柱(6/14)、肩关节(3/14)、骨盆(2/14)。13例患者单用或联合使用非甾体类抗炎药、糖皮质激素、改善病情的抗风湿药和肿瘤坏死因子-α(TNF-α)拮抗剂治疗,其中9例患者先后联合TNF-α拮抗剂治疗。对13例患者进行随访,9例患者单用或联合糖皮质激素、非甾体类抗炎药和DMARDs治疗后皮肤损害或骨关节肿痛症状控制欠佳,其中5例联合TNF-α抑制剂治疗后皮肤和骨关节症状均有不同程度改善,2例患者联合使用英夫利西单抗后皮疹加重后改为益赛普,1例拒绝治疗。结论本组SAPHO综合征男性多见,主要受累的骨关节是前胸壁,皮肤损害主要表现为掌跖脓疱病,且多见于女性,重度痤疮多见于男性。TNF-α拮抗剂对难治性患者有效,尤其是联合DMARDs能够快速控制骨关节病情,但可能会加重皮肤损害。针对本病建议采用多学科协作的诊疗模式,给予患者最佳诊疗方案,提高预后和生活质量。Objective To investigate the clinical features of SAPHO syndrome.Methods The clinical data, treatment and curative effect of 14 patients with SAPHO syndrome were analyzed retrospectively.Results There were 8 males and 6 females with an average age of onset of(28.43 ± 13.38) years. All 14 patients had skin lesions(8 palmoplantar pustulosis, 2 acne vulgaris, 3 cystic acne, and 1 acne conglobata).The bone and joint involved in the lesions in imaging were the anterior chest wall(13/14), sacroiliac joint(11/14), limb bone and joint(6/14), spine(6/14), shoulder joint(3/14), and pelvis(2/14).Thirteen patients were treated with non-steroidal anti-inflammatory drugs, glucocorticoids, disease-modifying anti-rheumatic drugs and tumor necrosis factor-α(TNF-α) antagonists, of which 9 patients were successively treated with TNF-α antagonists.Thirteen patients were followed up. Nine patients had poor control of skin lesions or bone and joint swelling and pain symptoms after treatment with glucocorticoids, non-steroidal anti-inflammatory drugs and DMARDs, of which 5 patients had different degrees of improvement of skin and bone and joint symptoms after treatment with TNF-α inhibitors, 2 patients were changed to Yisaipu after aggravation of rash after combined use of infliximab, and 1 patient refused treatment.Conclusion In this group, SAPHO syndrome is predominantly male. The main bone and joint involved is the anterior chest wall. The main skin lesions are palmoplantar pustulosis, which is more common in females, while severe acne is more common in males. TNF-α antagonists are effective in refractory patients, especially in combination with DMARDs to rapidly control osteoarticular disease, but may aggravate skin lesions. It is recommended to provide a multidisciplinary collaborative diagnosis and treatment model for patients to improve the prognosis and quality of life.

关 键 词：SAPHO综合征 掌跖脓疱病 痤疮 骨关节炎 TNF-α拮抗剂 

分 类 号：R753.1[医药卫生—皮肤病学与性病学]