Division of Rheumatology, Department of Internal Medicine, Patras University Hospital, University of Patras Medical School, Patras, Greece.
Division of Rheumatology, Department of Internal Medicine, Patras University Hospital, University of Patras Medical School, Patras, Greece.
Semin Arthritis Rheum. 2019 Feb;48(4):618-625. doi: 10.1016/j.semarthrit.2018.04.003. Epub 2018 Apr 17.
The SAPHO syndrome is a relatively rare clinical entity characterized by a wide range of dermatological and musculoskeletal manifestations. Biologics have been used in cases refractory to conventional treatment.
We present herein a patient with refractory to treatment SAPHO syndrome who exhibited a dramatic and fast response to IL-17 blockade. Additionally, we performed a systematic review of all cases of patients with SAPHO syndrome treated with biologics to date.
We identified 66 cases treated with biologics (45 with TNF blockers, 7 with IL-1 blockers, 13 with biologics targeting the IL-23/IL-17 axis, and 1 with tocilizumab). Data support a positive effect of anti-TNF treatment in SAPHO with a response rate in bone and joint manifestations of 93.3%. Skin disease also improved in 21/29 cases (72.4%). Data related to IL-1 inhibition in SAPHO are encouraging with most patients exhibiting a significant response in musculoskeletal manifestations (6/7, 85.7%). However, IL-1 inhibition is not effective in skin manifestations. Ustekinumab seems to have some efficacy with 2/4 patients responding in skin and 3/5 in bone/joint manifestations. Data related to IL-17 blockade indicate efficacy in skin disease with 4/7 patients responding (57.1%). Joint/bone manifestations improved in 3/8 patients (37.5%).
In SAPHO patients not responding to conventional treatment, TNF blockers appear to be the first choice. In patients failing TNF blockers, IL-1 inhibitors and biologics targeting the IL-17/IL-23 axis could be used.
SAPHO 综合征是一种相对罕见的临床实体，具有广泛的皮肤和肌肉骨骼表现。生物制剂已被用于常规治疗无效的病例。
我们在此介绍一例对治疗有反应的 SAPHO 综合征患者，其对白细胞介素 17 阻断剂反应迅速。此外，我们对迄今为止所有接受生物制剂治疗的 SAPHO 综合征患者的病例进行了系统回顾。
我们共确定了 66 例接受生物制剂治疗的病例（45 例接受 TNF 阻滞剂治疗，7 例接受白细胞介素 1 阻滞剂治疗，13 例接受针对白细胞介素 23/白细胞介素 17 轴的生物制剂治疗，1 例接受托珠单抗治疗）。数据支持抗 TNF 治疗在 SAPHO 中的积极作用，骨骼和关节表现的缓解率为 93.3%。21/29 例（72.4%）皮肤疾病也有所改善。与 SAPHO 相关的白细胞介素 1 抑制的数据令人鼓舞，大多数患者的肌肉骨骼表现有显著改善（6/7，85.7%）。然而，白细胞介素 1 抑制对皮肤表现无效。乌司奴单抗似乎有一定疗效，4/4 例患者的皮肤和 3/5 例患者的骨骼/关节表现有反应。与白细胞介素 17 阻断剂相关的数据表明，皮肤疾病的疗效为 4/7 例患者（57.1%）。3/8 例患者（37.5%）关节/骨骼表现改善。
在对常规治疗无反应的 SAPHO 患者中，TNF 阻滞剂似乎是首选。在 TNF 阻滞剂治疗失败的患者中，可以使用白细胞介素 1 抑制剂和针对白细胞介素 17/白细胞介素 23 轴的生物制剂。
