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8. An autoinflammatory syndrome resembling Castleman’s disease with excellent response to IL-6 blockade

机译:8.类似于卡斯尔曼病的自发性炎症综合症对IL-6阻断反应出色

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摘要

IntroductionAutoinflammatory diseases are an emerging group, characterised by recurrent inflammatory episodes due to dysregulated innate immunity. Common features include fevers, rash, arthralgia, lymphadenopathy and systemic symptoms.Castleman’s disease is a rare lymphoproliferative disease, associated with the overproduction of interleukin-6 (IL-6). Its two main variants: unicentric and multicentric differ in aetiology and clinical outcomes. The cytokine storm driven by IL-6 can mimic autoinflammatory disease.We present the case of an acquired autoinflammatory syndrome in a 33-year-old male with the clinical phenotype of Castleman’s but no culprit lymph node detected radiologically. Symptoms dramatically improved with yocilizumab, an IL-6 blocker.
机译:引言自体炎症是一种新兴疾病,其特征是先天免疫功能失调导致反复发炎。常见特征包括发烧,皮疹,关节痛,淋巴结肿大和全身症状。Castleman病是一种罕见的淋巴增生性疾病,与白细胞介素6(IL-6)的过量产生有关。其两个主要变体:单中心性和多中心性在病因和临床结局方面有所不同。由IL-6引起的细胞因子风暴可以模拟自身炎症性疾病。我们介绍了一名33岁男性患有后天性自身炎症综合症的病例,该男性的临床表型为Castleman氏病,但放射学未发现罪魁祸首。用IL-6阻断剂yocilizumab可以显着改善症状。

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