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系统性红斑狼疮背景下的最后区综合征:病例报告

Postrema area syndrome in the context of systemic lupus erythematosus: Case report.

作者信息

Muñoz-Zúñiga Jose Ricardo, Tamayo-Delgado Andrea Del Mar, Shinchi-Tanaka Alberto Masaru, Márquez Juan Camilo, Echeverri-García Alex, Garcia Santiago Lopez

机构信息

Residente de Neurología, Universidad Icesi, Facultad de Ciencias de la Salud, Cali, Colombia.

Residente de Radiología e imágenes diagnósticas, Universidad Icesi, Facultad de Ciencias de la Salud, Cali, Colombia.

出版信息

Heliyon. 2024 Nov 21;11(1):e40614. doi: 10.1016/j.heliyon.2024.e40614. eCollection 2025 Jan 15.

Abstract

INTRODUCTION

The area postrema, located on the floor of the fourth ventricle, regulates vomiting, fluid balance, osmoregulation, and immunomodulation. First documented in 1896, it has been a subject of scientific interest ever since. Area postrema syndrome (APS) is characterised by intractable nausea, vomiting, or hiccups, typically associated with neuromyelitis optica spectrum disorder (NMOSD). This paper presents a case of APS related with autoimmunity due to systemic lupus erythematosus (SLE) and not related to NMOSD. Additionally, a comprehensive review of the literature is conducted.

CASE REPORT

A 27-year-old Colombian female with a five-year history of systemic lupus erythematosus (SLE) presented with vomiting, epigastric pain, dysphagia, and seizures. Initial tests suggested acute pancreatitis and a lupus flare, but imaging of the gastrointestinal tract showed no abnormalities. Brain MRI revealed a lesion in the area postrema, indicating area postrema syndrome (APS). Treatment with hydrocortisone and later high-dose methylprednisolone and cyclophosphamide led to improvement. Negative anti-aquaporin 4 antibodies ruled out neuromyelitis optica spectrum disorder (NMOSD), leading to a probable diagnosis of APS associated with SLE.

CONCLUSION

Given the patient's negative AQP4-IgG results, clinical profile, and medical history, we propose APS associated with SLE, marking the first reported case in Latin America.

摘要

引言

最后区位于第四脑室底部,可调节呕吐、体液平衡、渗透压调节和免疫调节。它于1896年首次被记录,此后一直是科学研究的热点。最后区综合征(APS)的特征是顽固性恶心、呕吐或打嗝,通常与视神经脊髓炎谱系障碍(NMOSD)相关。本文介绍了一例因系统性红斑狼疮(SLE)导致的与自身免疫相关而非与NMOSD相关的APS病例。此外,还对文献进行了全面综述。

病例报告

一名27岁的哥伦比亚女性,有五年系统性红斑狼疮(SLE)病史,出现呕吐、上腹部疼痛、吞咽困难和癫痫发作。初步检查提示急性胰腺炎和狼疮发作,但胃肠道影像学检查未发现异常。脑部磁共振成像(MRI)显示最后区有病变,提示最后区综合征(APS)。使用氢化可的松治疗,随后使用大剂量甲泼尼龙和环磷酰胺治疗后病情好转。抗水通道蛋白4抗体阴性排除了视神经脊髓炎谱系障碍(NMOSD),从而可能诊断为与SLE相关的APS。

结论

鉴于患者抗水通道蛋白4免疫球蛋白G(AQP4-IgG)结果为阴性、临床特征和病史,我们提出与SLE相关的APS,这是拉丁美洲首例报告病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1879/11719383/025b0eda339f/gr1.jpg

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