Meryem Demir,Vasileios Kouranos,Kostadin Stoenchev,Stephen R Durham,Guy W Scadding,Nilesh Morar,Chadwan Al Yaghchi,Guri S Sandhu,Hesham Saleh
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引用次数: 0
Abstract
Sarcoidosis is an inflammatory, non-caseating granulomatous multisystem disease associated with JAK-STAT (Janus kinases-signal transducer and activator of transcription proteins) pathway activation. We present a patient with severe multi-systemic sarcoidosis who showed marked improvement with tofacitinib with regards to pulmonary, cutaneous, nasal and laryngeal disease. Tofacitinib prevented critical laryngeal stenosis from progressing to tracheostomy, induced regression of cutaneous lesions and improved pulmonary function in this steroid-resistant and immunosuppressive intolerant case. This case report supports further the role of JAK-inhibitors in the treatment of systemic sarcoidosis. Laryngoscope, 2024.
肉样瘤病是一种炎症性、非酪氨酸肉芽肿性多系统疾病，与JAK-STAT（Janus激酶-信号转导和激活转录蛋白）通路激活有关。我们介绍了一名患有严重多系统肉样瘤病的患者，该患者使用托法替尼后，肺部、皮肤、鼻腔和喉部疾病均有明显改善。托法替尼避免了严重的喉部狭窄发展到气管切开，促使皮肤病变消退，并改善了这位类固醇耐药和免疫抑制剂不耐受病例的肺功能。本病例报告进一步证实了 JAK 抑制剂在治疗系统性肉样瘤病中的作用。喉镜》，2024 年。
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