Syed Ameena, Shaik Sajid, Afshan Roshan, Karam Andrew, Hafeez Wasif, Almansour Sarmad
Department of Internal Medicine, Orlando Health Physician Associates, Orlando, USA.
Department of Internal Medicine, Saint Vincent Hospital, Worcester, USA.
Cureus. 2024 Jul 28;16(7):e65593. doi: 10.7759/cureus.65593. eCollection 2024 Jul.
Systemic lupus erythematosus (SLE) is a chronic inflammatory, multisystem autoimmune disease with a broad spectrum of clinical presentations. Neuropsychiatric systemic lupus erythematosus (NPSLE) refers to neurological and psychiatric symptoms involving the central and peripheral nervous systems. A 23-year-old African American female with a history of undifferentiated connective tissue disease on hydroxychloroquine and poor medication adherence presented to the emergency department with an altered mental status and generalized headache. In addition, she had a fever, associated tachycardia (104 BPM), and hypotension (90/63 mmHg). She was given fluids and started on broad-spectrum antibiotics and antivirals, suspecting bacterial or viral meningitis. However, a broad infectious workup, including cerebral spinal fluid (CSF) culture, was unrevealing. Given the lack of improvement of antibiotics, an immunological workup for SLE was initiated, which showed low CH50, C3, and C4; anti-nucleic acid antibody (ANA) was 1:1280, anti-double-stranded (anti-DS) DNA antibody not detected, and fluorescent ANA was positive. For severe NPSLE, rituximab is the most commonly utilized immunosuppressant; it was not utilized in this case due to the patient's insurance. The patient was placed on methylprednisolone and cyclophosphamide (CYC) infusion per ACR guidelines. Due to the toxic effects of CYC on the gonads, we offered ovarian preservation; however, the patient opted to refuse. The patient's mental status started to improve after three days of pulse steroids. The patient was advised to follow up with rheumatology for CYC therapy and a gradual taper of her steroids. NPSLE is a diagnosis of exclusion primarily based on expert opinion due to the absence of a gold standard diagnostic procedure. Disease-specific therapy, symptomatic therapy, nonpharmacological approaches, and correction of aggravating variables are all used to treat individuals with NPSLE. This paper aims to contribute to the existing literature on NPSLE, with the intention to educate and strive for early detection and treatment. We hereby present an interesting case of SLE in a 23-year-old female who would not have responded to one treatment. Instead, she needed multidisciplinary management, along with poor compliance.
系统性红斑狼疮（SLE）是一种慢性炎症性多系统自身免疫性疾病，临床表现多样。神经精神性系统性红斑狼疮（NPSLE）是指累及中枢和外周神经系统的神经和精神症状。一名23岁的非裔美国女性，有未分化结缔组织病病史，正在服用羟氯喹且用药依从性差，因精神状态改变和全身头痛就诊于急诊科。此外，她还发热，伴有心动过速（104次/分）和低血压（90/63 mmHg）。怀疑为细菌性或病毒性脑膜炎，给予补液并开始使用广谱抗生素和抗病毒药物。然而，包括脑脊液（CSF）培养在内的全面感染检查未发现异常。鉴于抗生素治疗无效，启动了针对SLE的免疫学检查，结果显示CH50、C3和C4水平低；抗核酸抗体（ANA）为1:1280，未检测到抗双链（抗-DS）DNA抗体，荧光ANA呈阳性。对于重症NPSLE，利妥昔单抗是最常用的免疫抑制剂；由于患者的保险问题，本病例未使用。根据美国风湿病学会（ACR）指南，该患者接受了甲泼尼龙和环磷酰胺（CYC）输注治疗。由于CYC对性腺有毒性作用，我们提供了卵巢保留方案；然而，患者选择拒绝。脉冲类固醇治疗三天后，患者的精神状态开始改善。建议患者随访风湿病科，接受CYC治疗并逐渐减少类固醇用量。由于缺乏金标准诊断程序，NPSLE主要基于专家意见进行排除性诊断。针对NPSLE患者的治疗包括疾病特异性治疗、对症治疗、非药物治疗方法以及纠正加重因素。本文旨在为现有的关于NPSLE的文献做出贡献，旨在进行教育并努力实现早期发现和治疗。我们在此呈现一例23岁女性SLE的有趣病例，该患者对一种治疗方法无反应。相反，她需要多学科管理，且依从性差。
