Department of Internal Medicine and Specialist Sciences, Institute of Dermatology, A. Gemelli University Hospital, Catholic University, Rome, Italy.
Am J Clin Dermatol. 2012 Feb 1;13(1):55-9. doi: 10.2165/11593250-000000000-00000.
SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome defines an association of inflammatory cutaneous disorders with osteoarticular manifestations and represents a clinical and therapeutic challenge. We report a case of severe SAPHO syndrome with acne conglobata and a diffuse involvement of the anterior chest wall and sacroiliac joints that required treatment with isotretinoin and adalimumab, a new fully human anti-tumor necrosis factor (TNF)-α monoclonal antibody. Combination treatment determined a complete clinical remission of cutaneous and osteoarticular manifestations after 48 weeks. Despite maintenance of clinical remission, follow-up imaging studies after 24 months of adalimumab monotherapy revealed osteoarticular disease progression, with features of inflammatory osteitis. TNFα antagonists have been used as third-line therapy for SAPHO syndrome in single case reports or case series, but these lack consistent long-term follow-up. SAPHO syndrome can present an intermittent-favorable course in the majority of cases as well as a chronic-progressive course, the latter requiring aggressive combination treatment with TNFα antagonists and conventional systemic agents.
SAPHO（滑膜炎-痤疮-脓疱病-骨肥厚-骨炎）综合征定义为炎症性皮肤疾病与骨关节表现的关联，代表了临床和治疗方面的挑战。我们报告了一例严重的 SAPHO 综合征，伴聚合性痤疮和前胸部及骶髂关节广泛受累，需要异维 A 酸和阿达木单抗（一种新型全人源抗肿瘤坏死因子（TNF）-α单克隆抗体）治疗。联合治疗在 48 周后使皮肤和骨关节炎表现完全缓解。尽管维持临床缓解，但阿达木单抗单药治疗 24 个月后的随访影像学研究显示骨关节炎疾病进展，具有炎症性骨炎的特征。TNFα 拮抗剂已在个案报告或病例系列中被用作 SAPHO 综合征的三线治疗药物，但这些研究缺乏一致的长期随访。SAPHO 综合征在大多数情况下呈间歇性-有利病程，也有慢性-进展性病程，后者需要 TNFα 拮抗剂和常规全身药物的强化联合治疗。
