2019 年第 2 期 第 14 卷

SAPHO综合征临床特点分析

Clinical characteristics of SAPHO syndrome

作者：田真李霞李赫李小霞赵义

英文作者：

单位：100053北京，首都医科大学宣武医院风湿免疫科

英文单位：

关键词：SAPHO综合征；掌跖脓疱病；痤疮；骨炎；肿瘤坏死因子α抑制剂

英文关键词：

• 摘要：

• 【摘要】目的    分析SAPHO综合征的临床特点。方法    收集2012年3月至2018年3月首都医科大学宣武医院诊断为SAPHO综合征的17例患者的临床资料、治疗方案及疗效情况进行回顾性分析，并对预后进行随访。结果    17例患者中男10例、女7例，平均诊断年龄为53岁（19～73岁）。有15例（88.2%）出现皮肤受累表现，主要表现为掌跖脓疱病、银屑病和痤疮。骨关节最常受累位点为前胸壁，有12例（70.6%），其次为脊柱6例、骶髂关节5例和外周关节3例。皮肤表现多与骨关节症状同时出现（9例，52.9%），但也可早于或晚于骨关节症状出现。疾病发作期多有红细胞沉降率升高（8例，47.1%）、C反应蛋白水平升高（10例，58.8%），自身抗体均为阴性，仅有1例患者（5.9%）为人类白细胞抗原B27阳性。非甾体消炎药及小剂量糖皮质激素在大多数患者急性发作期有效，但缓解期均需抗风湿药物进行维持。常用治疗药物包括甲氨蝶呤、来氟米特、雷公藤多苷、柳氮磺吡啶、双膦酸盐类药物。有3例患者（17.6%）在疾病复发后接受了肿瘤坏死因子α抑制剂的治疗，取得较好效果，随访2年未复发。结论    SAPHO综合征是一种以皮肤和骨关节为主要临床表现的血清阴性免疫炎症性疾病，急性期应积极抗炎治疗，后期需要改善病情使用抗风湿药物维持治疗，对于病情严重、复发的患者使用肿瘤坏死因子α抑制剂治疗有较好的效果。

• 【Abstract】Objective    To investigate the clinical features of SAPHO syndrome(synovitis, acne, pustulosis, hyperostosis, osteomyelitis syndrome). Methods    From March 2012 to March 2018, 17 patients were diagnosed of SAPHO syndrome at Xuanwu Hospital, Capital Medical University. Medical data, treatment plans and outcomes were retrospectively analyzed. Results    There were 10 males and 7 females. The average age was 53（19-73） years. Fifteen patients（88.2%）had skin involvements, mainly manifesting as palmoplantar pustulosis, psoriasis and acne. Skeletal lesions mainly involved anterior chest wall(n＝12, 70.6%), spine(n＝6), sacroiliac joint(n＝5) and peripheral joint(n＝3). Nine patients(52.9%) developed skin and osteoarticular manifestations simultaneously. Laboratory examination showed high erythrocyte sedimentation rate（n＝8, 47.1%） and C-reactive protein level（n＝10, 58.8%）; autoantibody was negative; only 1 patient（5.9%） showed leucocyte antigen B27(＋). In the acute stage of disease, non-steroid anti-inflammatory drugs and low dose corticosteroids were effective in most patients. Antirheumatic drugs were needed in maintaining remission. Therapeutic drugs included methotrexate, leflunomide, tripterygium wilfordii, sulfasalazine and bisphosphonates. Three patients（17.6%） were treated with tumor necrosis factor-α inhibitors after recurrence; all of them showed good response and had no relapse in 2 years. Conclusions    SAPHO syndrome is a seronegative inflammatory disease characterized by dermatological and osteoarticular lesions. Anti-inflammatory drugs should be used in the acute period and antirheumatic drugs are necessary in the remission stage. Tumor necrosis factor-α inhibitors have a good therapeutic effect on patients with severe condition and relapse.