Muñoz-Zúñiga Jose Ricardo, Tamayo-Delgado Andrea Del Mar, Shinchi-Tanaka Alberto Masaru, Márquez Juan Camilo, Echeverri-García Alex, Garcia Santiago Lopez
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.
Heliyon. 2024 Nov 21;11(1):e40614. doi: 10.1016/j.heliyon.2024.e40614. eCollection 2025 Jan 15.
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.
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.
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.
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。
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。
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。