Schnitzler-Like Syndrome Presenting With IgG Kappa Monoclonal Gammopathy: A Case Report and Review of Diagnostic and Therapeutic Challenges.

Schnitzler 样综合征表现为 IgG κ 单克隆 γ 病 ： 病例报告以及诊断和治疗挑战的回顾。

• 发表时间: 2024 Jul
• 来源期刊: Cureus
• DOI: 10.7759/cureus.64440
• PMID: 39135817
• 文章类型: Case Reports
• 作者列表: Singh G,Goswami K,Trehan S,Kachhadia MP,Farooq A,Puri P,Azhar W

关键词: autoimmune disease; autoinflammatory disorder; case report; chronic urticarial rash; igg kappa; joint pain; monoclonal gammopathy; schnitzler syndrome; schnitzler-like syndrome; systemic inflammation

Abstract

Schnitzler syndrome (SS) is a rare autoinflammatory disorder characterized by a constellation of symptoms that include chronic urticarial rash, recurrent fever, arthralgias/arthritis, and monoclonal gammopathy, typically involving immunoglobulin M (IgM). However, cases with overlapping clinical features but lacking specific criteria fall under the umbrella of Schnitzler-like syndromes. This case report describes a 40-year-old male with Schnitzer-like syndrome and underscores the diagnostic complexities and therapeutic challenges of Schnitzer-like syndrome with IgG kappa monoclonal gammopathy, highlighting the need for a comprehensive diagnostic approach and targeted therapy.

摘要

Schnitzler综合征（SS）是一种罕见的自身炎症性疾病，其特征是一系列症状，包括慢性荨麻疹。反复发烧,关节痛/关节炎,和单克隆丙种球蛋白病，通常涉及免疫球蛋白M（IgM）。然而,临床特征重叠但缺乏特定标准的病例属于Schnitzler样综合征。该病例报告描述了一名40岁的男性，患有Schnitzer样综合征，并强调了具有IgGκ单克隆丙种球蛋白病的Schnitzer样综合征的诊断复杂性和治疗挑战。强调需要全面的诊断方法和靶向治疗。