无单克隆γ病的Schnitzler综合征:系统回顾和病例报告。

IF 2.8 4区 医学 Q1 DERMATOLOGY
Catherine Zhu, Elvis Martinez-Jaramillo, Moshe Ben Shoshan, Elena Netchiporouk, May Chergui, Michael Fein
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引用次数: 0

摘要

背景:Schnitzler综合征(SS)是一种罕见的自身炎症性疾病,表现为慢性荨麻疹、中性粒细胞性荨麻疹皮肤病、单克隆IgG或IgM γ病、复发性发热、关节和/或骨痛、淋巴结、脾脏和/或肝脏肿大。文献中有几个病例描述了缺乏单克隆γ病的SS的不完全变异。本系统综述旨在分析目前文献中有关施尼茨勒样综合征(Schnitzler-like Syndrome, SLS)的特点及其治疗效果;我们也报告了我们自己的SLS病例。方法:系统检索MEDLINE、Embase、Scopus和PubMed (PROSPERO: CRD42024548245)。所有英文或法文文章均被纳入,且不受出版日期限制。所有具有原始数据、临床特征和治疗结果的文章均被纳入。两名审稿人独立进行筛选,如有必要,由第三名审稿人解决冲突。结果:15项研究(13例病例报告和2例病例系列)符合纳入标准,另外还有1例来自我们自己的经验,共计18例患者。大多数患者为男性(55.6%),诊断时的中位年龄为57.5岁(IQR=44.8, 62.8)。我们的大多数患者没有单克隆伽玛病(n = 14, 77.8%), 4例(22.2%)有迟发性单克隆伽玛病。抗组胺药是最常见的治疗方法(n=17; 94.4%),其次是阿那单抗(n=14; 77.8%)、强的松(n= 9; 50.0%)、未指定全体性皮质激素(n=8; 44.4%)、奥玛珠单抗(n=8; 44.4%)、秋水仙碱(n= 5; 27.8%)、氨苯松(n= 6; 33.3%)、环孢素(n=4; 22.2%)、canakinumab (n=4; 22.2%)、羟氯喹(n=2; 11.1%)和沙利度胺(n=1; 5.6%)。在接受anakinra治疗的14例患者中,92.9% (n = 13/14)达到完全缓解(CR)。结论:我们的研究结果提出了单克隆γ病可能不是施尼茨勒综合征的强制性标准的假设。我们建议将此作为未来共识讨论的考虑因素,强调早期识别和及时使用IL-1抑制剂治疗以改善患者预后的潜在益处。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Schnitzler Syndrome without Monoclonal Gammopathy: A systematic review and case presentation.

Background: Schnitzler syndrome (SS) is a rare autoinflammatory disorder that presents with chronic urticarial rash, neutrophilic urticarial dermatosis, monoclonal IgG or IgM gammopathy, recurrent fever, joint and/or bone pain and enlarged lymph nodes, spleen, and/or liver. Several cases in the literature have described incomplete variants of SS that lack monoclonal gammopathy. This systematic review aims to analyze the current literature regarding the features of Schnitzler-like Syndrome (SLS) and their treatment outcomes; we also report our own case of SLS.

Methods: A systematic search of MEDLINE, Embase, Scopus and PubMed was performed (PROSPERO: CRD42024548245). All articles in English or French were included, and no publication date restrictions were applied. All articles with original data, clinical features and treatment outcomes were included. Two reviewers independently conducted screenings and conflicts were resolved by a third reviewer if necessary.

Results: 15 studies (13 case reports and 2 case series) met inclusion criteria, in addition to one case from our own experience, yielding a total of 18 patients. The majority of our population was male (55.6%) with a median age at diagnosis of 57.5 (IQR=44.8, 62.8). The majority of our patients had absent monoclonal gammopathy (n = 14; 77.8%), and four (22.2%) had delayed-onset monoclonal gammopathy. Antihistamines were the most common treatment (n=17; 94.4%), followed by anakinra (n=14; 77.8%), prednisone (n = 9; 50.0%), unspecified systemic corticosteroids (n=8; 44.4%), omalizumab (n=8; 44.4%), colchicine (n = 5; 27.8%), dapsone (n = 6; 33.3%), cyclosporine (n = 4; 22.2%), canakinumab (n=4; 22.2%), hydroxychloroquine (n=2; 11.1%) and thalidomide (n=1; 5.6%). Of the 14 patients treated with anakinra, 92.9% (n = 13/14) achieved a complete response (CR).

Conclusions: Our findings raise the hypothesis that monoclonal gammopathy may not be an obligate criterion for Schnitzler syndrome. We propose this as a consideration for future consensus discussions, emphasizing the potential benefits of early recognition and timely IL-1 inhibitor therapy to improve patient outcomes.

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来源期刊
CiteScore
3.20
自引率
2.40%
发文量
389
审稿时长
3-8 weeks
期刊介绍: Clinical and Experimental Dermatology (CED) is a unique provider of relevant and educational material for practising clinicians and dermatological researchers. We support continuing professional development (CPD) of dermatology specialists to advance the understanding, management and treatment of skin disease in order to improve patient outcomes.
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