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伴有IgG κ单克隆丙种球蛋白病的施尼茨勒样综合征:一例报告及诊断与治疗挑战的综述

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

作者信息

Singh Gurjot, Goswami Kanishka, Trehan Shubam, Kachhadia Meet P, Farooq Amna, Puri Piyush, Azhar Waqas

机构信息

Internal Medicine, Southern Illinois University School of Medicine, Springfield, USA.

Internal Medicine, Memorial Medical Center, Springfield, USA.

出版信息

Cureus. 2024 Jul 12;16(7):e64440. doi: 10.7759/cureus.64440. eCollection 2024 Jul.

DOI:10.7759/cureus.64440
PMID:39135817
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11317788/
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

施尼茨勒综合征(SS)是一种罕见的自身炎症性疾病,其特征是一系列症状,包括慢性荨麻疹皮疹、反复发热、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,具有重叠临床特征但缺乏特定标准的病例属于施尼茨勒样综合征的范畴。本病例报告描述了一名患有施尼茨勒样综合征的40岁男性,并强调了伴有IgG κ单克隆丙种球蛋白病的施尼茨勒样综合征的诊断复杂性和治疗挑战,突出了全面诊断方法和靶向治疗的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9bd/11317788/9d2e98b1497d/cureus-0016-00000064440-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9bd/11317788/db473805b017/cureus-0016-00000064440-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9bd/11317788/9d2e98b1497d/cureus-0016-00000064440-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9bd/11317788/db473805b017/cureus-0016-00000064440-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9bd/11317788/9d2e98b1497d/cureus-0016-00000064440-i02.jpg

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1
Schnitzler-Like Syndrome Presenting With IgG Kappa Monoclonal Gammopathy: A Case Report and Review of Diagnostic and Therapeutic Challenges.伴有IgG κ单克隆丙种球蛋白病的施尼茨勒样综合征:一例报告及诊断与治疗挑战的综述
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本文引用的文献

1
Schnitzler Syndrome: Insights into Its Pathogenesis, Clinical Manifestations, and Current Management.施尼茨勒综合征:发病机制、临床表现及当前治疗方法的研究进展。
Biomolecules. 2024 May 31;14(6):646. doi: 10.3390/biom14060646.
2
Proposal of a new clinical entity: Paraprotein negative IL-1 mediated inflammatory dermatosis (PANID) that may precede Schnitzler syndrome.一种新的临床实体的提议:副蛋白阴性IL-1介导的炎症性皮肤病(PANID),可能先于施尼茨勒综合征出现。
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Tocilizumab treatment in patients with Schnitzler syndrome: An open-label study.
托珠单抗治疗施尼茨勒综合征患者:一项开放标签研究。
J Allergy Clin Immunol Pract. 2021 Jun;9(6):2486-2489.e4. doi: 10.1016/j.jaip.2021.01.024. Epub 2021 Feb 2.
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Schnitzler syndrome, an autoimmune-autoinflammatory syndrome: report of two new cases and review of the literature.施尼茨勒综合征,一种自身免疫-炎症性疾病:两例新病例报告及文献复习。
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The Schnitzler syndrome. Schnitzler 综合征。
Orphanet J Rare Dis. 2010 Dec 8;5:38. doi: 10.1186/1750-1172-5-38.
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Treatment of Schnitzler's syndrome with anakinra: report of three cases and review of the literature.用阿那白滞素治疗施尼茨勒综合征:三例报告及文献复习
Scand J Rheumatol. 2011 Jan;40(1):74-9. doi: 10.3109/03009742.2010.493894. Epub 2010 Oct 11.
10
Dramatic response to IL1-RA treatment in longstanding multidrug resistant Schnitzler's syndrome: a case report and literature review.对长期多药耐药 Schnitzler 综合征白细胞介素 1 受体拮抗剂治疗的显著反应:病例报告及文献复习。
Clin Rheumatol. 2010 May;29(5):567-71. doi: 10.1007/s10067-010-1375-9. Epub 2010 Feb 1.