JAK抑制剂在SAPHO综合征中的应用

Update of JAK Inhibitors in the Treatment of SAPHO Syndrome

Janus激酶-信号转导及转录激活因子(Janus kinase-signal transducer and activator of transcription,JAK-STAT)信号通路是一条介导多种细胞因子及生长因子信号转导的重要通路。SAPHO综合征是一种以滑膜炎(synovitis)、痤疮(acne)、脓疱病(pustulosis)、骨肥厚(hyperostosis)及骨炎(osteitis)为主要表现的自身炎症性疾病,与多种炎性细胞因子的增多密切相关,如肿瘤坏死因子(tumor necrosis factor,TNF)α、白细胞介素(interleukin,IL)-1等。研究发现,TNF-α、IL-1等细胞因子可通过JAK-STAT信号通路介导炎症反应,导致疾病的发生,阻断该通路有望成为治疗SAPHO综合征的新靶点。目前已有多个临床研究显示,JAK抑制剂治疗SAPHO综合征有效,但其远期疗效及安全性需进一步研究。

Janus kinase-signal transducer and activator of transcription(JAK-STAT)signaling pathway is an important pathway that mediates the signal transduction of a variety of cytokines and growth factors.SAPHO syndrome is an autoinflammatory disease with synovitis,acne,pustulosis,hyperostosis,and osteitis as the main manifestations.The increase of a variety of inflammatory cytokines is closely related to it,such as tumor necrosis factor(TNF)α,Interleukin(IL)-1.It is found that cytokines such as TNF-αand IL-1 can mediate inflammatory response through JAK-STAT signaling pathway,leading to the occurrence of disease;therefore blocking this pathway is expected to become a new target for the treatment of SAPHO syndrome.At present,many clinical studies have shown that JAK inhibitors are effective in treating SAPHO syndrome,but their long-term efficacy and safety need further study.