SAPHO综合征并发葡萄膜炎1例,TNF抑制剂和JAKinib均有良好反应。

Ritasman Baisya, Meghna Gavali, Mudit Tyagi, Phani Kumar Devarasetti
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引用次数: 0

摘要

简介:SAPHO(滑膜炎、痤疮、脓疱病、骨质增生和骨炎)综合征是一种罕见的自身炎症性疾病,描述了皮肤、骨骼和关节的炎症表现,导致诊断困难和治疗挑战。病例:在这里,我们提出一个病例的年轻男性诊断为SAPHO综合征与骨关节和皮肤累及从他的生活的早期。他长期遭受诊断上的困难,因此没有得到充分的治疗。他对常规dmard的反应很小,但对TNF抑制剂(阿达木单抗)的反应很好。后来,他没有接受治疗,出现急性前葡萄膜炎,阿达木单抗和托法替尼也显著改善,尽管患者一直面临经济拮据的问题。结论:该病例的独特之处在于患者有多器官受累,包括骨关节系统、皮肤和眼睛。TNFi(阿达木单抗)和JAKinib(托法替尼)对所有器官都有良好的反应,该患者的生活质量得到了净改善。
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A Case of SAPHO Syndrome Complicated by Uveitis with Good Response to Both TNF Inhibitor and JAKinib.

Introduction: SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare autoinflammatory condition describing the constellation of inflammatory skin, bone, and joint manifestations which result in diagnostic difficulty and therapeutic challenge.

Case: Here, we present a case of a young male diagnosed with SAPHO syndrome with osteoarticular and cutaneous involvement from an early age in his life. He suffered diagnostic challenges for a long time and was hence inadequately treated. He had minimal response to conventional DMARDs but showed excellent response to TNF inhibitor (adalimumab). Later, he defaulted treatment and presented with acute anterior uveitis which was also dramatically improved with adalimumab and tofacitinib although financial constraint was always an issue for the patient.

Conclusion: The uniqueness of this case was that the patient had a multiorgan involvement including osteoarticular system, skin, and eye. Both TNFi (adalimumab) and JAKinib (tofacitinib) had a good response to all organs with a net improvement in the quality of life of this patient.

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