Neuropsychiatric Systemic Lupus Erythematosus (NPSLE): A Case Report and an Overview of the Diagnosis, Treatment Modalities, and Prognosis

Abstract

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.