副蛋白阴性il -1介导的炎症性皮肤病:无Gammopathy的schnitzler样综合征的最新进展

Rizwan Ahmad , Jean Dunne , Katie Ridge , Nicole Fagan , Niall Conlon
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引用次数: 0

摘要

施尼茨勒综合征(SchS)是一种罕见的自身炎症性疾病,以慢性荨麻疹和全身炎症为特征。必要的诊断标准包括单克隆IgM或IgG条带的存在,几乎所有病例都表现出对IL-1阻断的迅速反应。最近,没有旁蛋白的“Schnitzler-like”病例也有报道。虽然它们与原始同名综合征的确切关系尚不清楚,但这些病例具有相似的临床特征和对IL-1抑制的反应。诊断延迟在自身炎症综合征中很常见,识别潜在新发病例的必要性很重要。我们报告了一例47岁男性难治性荨麻疹、关节疼痛和系统性炎症,类似于SchS,但没有检测到副蛋白,与最近提出的副蛋白阴性il -1介导的炎症性皮肤病(PANID)一致。在常规治疗失败后,患者通过IL-1阻断实现了快速和持续的缓解。本病例强调了在伴有相关炎症特征的难治性荨麻疹患者中识别自身炎症综合征(包括PANID)的重要性。它还强调了IL-1抑制治疗试验的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Paraprotein-Negative IL-1–Mediated Inflammatory Dermatosis: An Update on Schnitzler-Like Syndrome in the Absence of a Gammopathy
Schnitzler syndrome (SchS) is a rare autoinflammatory condition characterized by chronic urticaria and systemic inflammation. Obligate diagnostic criteria include the presence of a monoclonal IgM or IgG band, with nearly all cases demonstrating a prompt response to IL-1 blockade. Recently, "Schnitzler-like" cases without a paraprotein have been reported. Although the exact nature of their relation to the original eponymous syndrome remains unclear, these cases share similar clinical features and response to IL-1 inhibition. Diagnostic delay is common in autoinflammatory syndromes, and the need to recognize potentially emerging cases is important. We present the case of a male aged 47 years with refractory urticaria, joint pain, and systemic inflammation resembling SchS but without detectable paraprotein, consistent with recently proposed paraprotein-negative IL-1–mediated inflammatory dermatosis (PANID). After failing conventional therapies, the patient achieved rapid and sustained remission with IL-1 blockade. This case underscores the importance of recognizing autoinflammatory syndromes, including PANID, in patients with refractory urticaria with associated inflammatory features. It also highlights the importance of a therapeutic trial of IL-1 inhibition.
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