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使用JAK激酶抑制剂巴瑞替尼成功治疗难治性滑膜炎、痤疮、脓疱病、骨肥厚和骨炎(SAPHO)综合征

Successful Treatment of Refractory Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome with Baricitinib, a Janus Kinase Inhibitor.

作者信息

Yang Jianqiu, Yuan Chunyu, Zhou Shengru, Teng Zhicheng, Li Min

机构信息

Department of Dermatology, Dushu Lake Hospital Affiliated to Soochow University (Medical Center of Soochow University), Suzhou, People's Republic of China.

Suzhou Medical College of Soochow University, Suzhou, People's Republic of China.

出版信息

Clin Cosmet Investig Dermatol. 2024 Mar 4;17:529-537. doi: 10.2147/CCID.S446468. eCollection 2024.

DOI:10.2147/CCID.S446468
PMID:38463557
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10921886/
Abstract

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome, a rare immune-mediated inflammatory disease, poses diagnostic and therapeutic challenges owing to its multi-system involvement, high heterogeneity, and lack of specific laboratory tests. Additionally, lacking evidence-based treatment recommendations, with the primary approach focusing on symptomatic relief. Herein, we report the case of a 32-year-old Chinese woman who presented with recurrent, generalized multiple osteoarticular pain lasting over one year and skin erythema pustulosis for 11 months. Traditional treatments, including non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, disease-modifying antirheumatic drugs (DMARDs), and other traditional approaches, yielded no significant effects. Despite the prior use of adalimumab and acitretin capsules, the treatment remained unsatisfying, especially regarding the skin lesions. Considering the complex pathogenesis of SAPHO syndrome, the patient was orally administered baricitinib (2 mg), a Janus kinase (JAK) inhibitor, twice daily. A notable improvement in both skin lesions and osteoarticular pain was observed within two weeks of treatment initiation. Subsequently, the dosage of baricitinib was halved and continued for an additional three months, during which regular follow-ups revealed neither disease recurrence nor adverse effects. Collectively, the successful treatment of refractory SAPHO syndrome with baricitinib presents a promising implication for addressing the therapeutic challenges of this rare autoimmune condition, offering a potential breakthrough in managing its complex manifestations.

摘要

滑膜炎、痤疮、脓疱病、骨肥厚和骨炎(SAPHO)综合征是一种罕见的免疫介导性炎症性疾病,由于其多系统受累、高度异质性以及缺乏特异性实验室检查,给诊断和治疗带来了挑战。此外,由于缺乏循证治疗建议,主要治疗方法侧重于缓解症状。在此,我们报告一例32岁中国女性病例,该患者反复出现全身性多关节疼痛持续一年以上,皮肤红斑脓疱病11个月。包括非甾体抗炎药(NSAIDs)、皮质类固醇、改善病情抗风湿药(DMARDs)及其他传统方法在内的传统治疗均无显著效果。尽管先前使用了阿达木单抗和阿维A胶囊,但治疗效果仍不理想,尤其是皮肤病变方面。考虑到SAPHO综合征复杂的发病机制,患者口服巴瑞替尼(2mg)——一种 Janus激酶(JAK)抑制剂,每日两次。治疗开始后两周内,皮肤病变和骨关节疼痛均有显著改善。随后,巴瑞替尼剂量减半并继续服用三个月,在此期间定期随访未发现疾病复发及不良反应。总体而言,巴瑞替尼成功治疗难治性SAPHO综合征为应对这种罕见自身免疫性疾病的治疗挑战带来了希望,为管理其复杂表现提供了潜在突破。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/754f/10921886/ef4a0fb786f2/CCID-17-529-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/754f/10921886/a951cd0fe28f/CCID-17-529-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/754f/10921886/ef4a0fb786f2/CCID-17-529-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/754f/10921886/a951cd0fe28f/CCID-17-529-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/754f/10921886/ef4a0fb786f2/CCID-17-529-g0002.jpg

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本文引用的文献

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Int J Rheum Dis. 2023 Mar;26(3):563-567. doi: 10.1111/1756-185X.14542. Epub 2022 Dec 20.