抗肿瘤坏死因子-α拮抗剂在SAPHO综合征治疗中的应用

摘要

目的 SAPHO综合征属罕见病,尚缺乏统一的治疗方案,本文旨在探讨抗肿瘤坏死因子-α拮抗剂在本病治疗中的作用.方法 对8例难治性SAPHO综合征患者的临床资料、治疗及转归进行分析并复习相关文献.结果 8例患者均为女性.7例表现为掌跖脓疱病,1例无皮肤损害.骨关节受累中上胸壁处受累8例,外周关节受累6例,骶髂关节受累6例,脊柱关节受累2例.8例患者均给予非甾体抗炎药(NSAID)、糖皮质激素和(或)改变病情抗风湿药(DMARD)和(或)双磷酸盐,症状均无改善,后加用抗肿瘤坏死因子-α拮抗剂,症状均改善.但在治疗过程中,1例出现皮疹加重,1例出现颌下腺炎；4例停药后复发；3例配合中药治疗,症状均不同程度改善.结论 抗肿瘤坏死因子-α可显著改善SAPHO综合征的症状,但个别患者出现皮疹加重,有潜在的感染风险,且停药后易复发；中医药治疗本病有一定作用,有望成为治疗本病的新方法.%Objective Synovitis, acne, pustulosis, hyperostosis, osteitis ( SAPHO) syndrome is a very rare disease and still lack standardized therapy. We analyzed the clinical efficacy of anti - tumor necrosis factor - α ( TNF -α) inhibitor therapy in treatment of SAPHO syndrome. Methods We analyzed 8 patients with refractory SAPHO syndrome treating with anti - TNF - α inhibitors, and reviewed cases treating with anti - TNF -α inhibitors reported in the literature. Results Eight patients were all female:7 of them had palmoplantar pustulosis, the other one was free of skin lesions. Their osteoarticular manifestations included anterior chest wall involving in 8 patients,peripheral joints involving in 6 patients, sacroiliac joints involving in 6 patients, and vertebral joints involving in 2 patients. All patients were treated with non - steroidal anti - inflammatory drugs ( NSAIDs) as the first - line therapy, and corticosteroid and ( or) disease modifying anti - rheumatic drugs ( DMARDs) as the second - line drug, but the symptoms cannot be ameliorated. Patients received anti - TNF - α inhibitors as the third - line therapy, and each of them had their symptoms improved. During the treatment of anti - TNF - α inhibitors, skin rash of 1 patient aggravated, and 1 patient developed submandibular sialadenitis. Four patients had disease relapse after cessation of anti - TNF - α inhibitor therapy. Three patients received Chinese traditional medicine as additional therapy, and had symptoms improved further. Conclusion Anti - TNF - α inhibitors can ameliorate symptoms of SAPHO syndrome patients significantly, but skin rash in some patients aggravates during the treatment. The risk of infection is the most potential side - effect of anti - TNF - α therapy. Relapse of disease is not unusual after the cessation of treatment. Chinese traditional medicine is proved to be valid in SAPHO syndrome, and would be developed into the potential new therapy of this rare disease.