Neuropsychiatric Lupus in Singapore: Disease Characteristics and Outcomes

Introduction:The aim of this study is to describe the clinical characteristics and outcomes of Systemic lupus erythematosus (SLE) patients with central nervous system (CNS) involvement in our population. Methods:We conducted a retrospective review of the medical records of patients diagnosed with neuropsychiatric lupus (NPSLE) in Singapore General Hospital between Jan 2007 and Oct 2017.Results: The records of eighteen patients were analysed retrospectively.The median age of diagnosis of NPSLE was 33.5 years.The median duration of SLE at the time of the neuropsychiatric manifestations was 4.5 months.Thirty-three percent had NPSLE as the first presentation of SLE.The most frequent clinical presentation was acute confusional state (30.8%) followed by cerebrovascular disease (23.1%) and seizures (19.2%).Seven patients (38.9%) had more than one neuropsychiatric syndrome.The commonest Magnetic Resonance Imaging (MRI) brain abnormality was white matter hyperintensities (61.1%), followed by acute infarcts (33.3%) and cerebral hemorrhage (22.2%).Multi-vessel involvement (3 or more vessels) (55.6%) was observed on magnetic resonance angiography (MRA).Majority of the patients (72.2%) received intravenous methylprednisolone, 66.7% had intravenous cyclophosphamide and 22.2% had rituximab.Twelve patients (66.7%) had clinical improvement and three patients (16.7%) had relapsing courses.There were three deaths from diffuse alveolar hemorrhage and ischaemic heart disease.Conclusion: Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) manifests in a variety of clinical presentations, with NPSLE syndromes being the first presentation of SLE in one third of the patients.Prompt diagnosis and management is essential to improve clinical outcomes.