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儿童期红斑狼疮综合征:全面的系统文献综述。

Rhupus syndrome in the pediatric population: A comprehensive systematic literature review.

机构信息

Universidad San Francisco de Quito, Quito, Ecuador.

出版信息

Medicine (Baltimore). 2024 Apr 5;103(14):e36451. doi: 10.1097/MD.0000000000036451.

Abstract

INTRODUCTION

The term "Rhupus" was employed to descriptively illustrate the overlap observed in some pediatric patients displaying features of both juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE). Although "Rhupus" is traditionally used in adults, we applied it broadly to emphasize this clinical overlap.

METHODS

We sought to identify studies that registered signs, symptoms, imaging characteristics, and treatments given to patients with JIA and SLE. We searched four databases using a Boolean search string, resulting in 231 articles after duplicate removal. Title and abstract screening yielded 57 articles for full-text assessment. Full reviewed 13 extracted data regarding sex, age of onset, serologic and imaging findings, and management strategies. The NIH quality assessment tool was applied to ensure the internal validity of the articles.

RESULTS

From the 13 articles evaluated that meet inclusion criteria, none had standardized diagnostic algorithms. The total number of patients in those articles is 26, without discussing treatment guidelines.

DISCUSSION

Clinical presentation, diagnostic parameters, and treatment of pediatric Rhupus were synthesized in this review. Fundamental keys help distinguish the joint presentation when Juvenile Idiopathic Arthritis or Lupus is present, compared with the signs and symptoms when developing the overlapping syndrome. We highlight the importance of physicians knowing about this rare condition and call all specialists to report new cases of the disease so a consensus can be reached to establish standardized guidelines for diagnosing and treating Rhupus syndrome.

摘要

简介

“Rhupus”一词被用来描述一些儿科患者表现出幼年特发性关节炎(JIA)和系统性红斑狼疮(SLE)特征的重叠现象。虽然“Rhupus”传统上用于成人,但我们广泛地使用它来强调这种临床重叠。

方法

我们试图确定登记了 JIA 和 SLE 患者的体征、症状、影像学特征和治疗方法的研究。我们使用布尔搜索字符串在四个数据库中进行搜索,删除重复项后得到 231 篇文章。标题和摘要筛选产生了 57 篇全文评估文章。全文评估了 13 篇提取的数据,涉及性别、发病年龄、血清学和影像学发现以及管理策略。应用 NIH 质量评估工具确保了文章的内部有效性。

结果

从符合纳入标准的 13 篇文章中,没有一篇有标准化的诊断算法。这些文章中的总患者人数为 26 人,没有讨论治疗指南。

讨论

本综述综合了儿科 Rhupus 的临床表现、诊断参数和治疗方法。当存在幼年特发性关节炎或狼疮时,关节表现的基本关键有助于区分,而在出现重叠综合征时,区分的是体征和症状。我们强调医生了解这种罕见疾病的重要性,并呼吁所有专家报告新病例,以便就诊断和治疗 Rhupus 综合征达成共识并制定标准化指南。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b73/10994449/62f7c0312d9f/medi-103-e36451-g001.jpg

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