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罗威尔综合征:关于一种罕见且具有挑战性疾病的发病机制及靶向治疗前景的综合综述

Rowell Syndrome: A Comprehensive Review on Pathogenesis of a Rare and Challenging Entity and the Horizon of Targeted Therapeutics.

作者信息

Khayyat Azadeh, Zohouri Seyed Amir, Esmaaeil Pour Mohammad Ali, Hatami Parvaneh, Smoller Bruce R

机构信息

Pathology Department Medical College of Wisconsin Milwaukee Wisconsin USA.

Science Department Simon Fraser University Burnaby British Columbia Canada.

出版信息

Health Sci Rep. 2025 Feb 17;8(2):e70470. doi: 10.1002/hsr2.70470. eCollection 2025 Feb.

Abstract

BACKGROUND

Rowell syndrome (RS), an infrequent illness characterized by a combination of erythema multiforme-like manifestations and systemic lupus erythematosus, poses diagnostic and clinical issues to the healthcare system. Initially discovered by Dr. Virginie Rowell in 1963, the disorder manifests as an overlapping illness with various dermatological and rheumatological conditions, necessitating precise assessment.

METHOD

The review encompasses demographic characters, dermatological signs and symptoms, triggering or contributing factors, laboratory studies, diagnosis and therapy challenges, and the outcome of RS. Through an analysis of the literature, this study identifies sunlight, medication use, COVID-19 vaccination, and bacterial infections as contributing predisposing risks. Laboratory abnormalities revealed positive antinuclear antibodies and rheumatoid factor as common features, supporting the autoimmune origin of this illness.

RESULT

Medications vary, with systemic corticosteroids used as the initial therapy, and appropriate proper outcomes are observed with rituximab and hydroxychloroquine. Recent discoveries in biological agents and targeted immunotherapies have provided useful options for personalized care.

CONCLUSION

In summary, our study provides detailed and valuable data to deepen our knowledge about RS, showing the need for further investigations to discover the mechanisms underlying its pathogenesis and to identify novel targeted agents that can individualize the treatment plan.

摘要

背景

罗威尔综合征(RS)是一种罕见疾病,其特征为多形红斑样表现与系统性红斑狼疮并存,给医疗系统带来了诊断和临床方面的问题。该疾病于1963年由维吉妮·罗威尔医生首次发现,表现为一种与多种皮肤病和风湿病相关的重叠性疾病,需要进行精确评估。

方法

该综述涵盖了人口统计学特征、皮肤体征和症状、触发或促成因素、实验室检查、诊断和治疗挑战以及RS的结局。通过对文献的分析,本研究确定阳光、药物使用、新冠病毒疫苗接种和细菌感染为促成易患风险因素。实验室异常结果显示抗核抗体和类风湿因子呈阳性是常见特征,支持了该疾病的自身免疫起源。

结果

用药方案各不相同,初始治疗使用全身用皮质类固醇,使用利妥昔单抗和羟氯喹可观察到适当的良好疗效。生物制剂和靶向免疫疗法方面的最新发现为个性化治疗提供了有益选择。

结论

总之,我们的研究提供了详细且有价值的数据,以加深我们对RS的了解,表明需要进一步开展研究以发现其发病机制背后的原理,并确定能够使治疗方案个性化的新型靶向药物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f92/11831000/0b479300c2d5/HSR2-8-e70470-g001.jpg

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