Abstract
Objective: Neuropsychiatric systemic lupus erythematosus (NPSLE) is a relatively common and potentially serious manifestation of SLE. This study was designed to collect evidence about clinical and demographic characteristics of patients with NPSLE in a Pakistani lupus cohort.
Methodology: This cross-sectional study was conducted at Department of Rheumatology, Pakistan Institute of Medical Sciences, Islamabad, Pakistan between July 2016 and December 2016. Patients fulfilling diagnostic criteria for SLE as defined by ACR were enrolled. For detection of neuropsychiatric involvement the One-Hour Neuropsychological Battery proposed by the ACR was performed. Neuropsychiatric manifestations were classified into major and minor. Relationship of presence and severity of individual neuropsychiatric manifestations to disease duration in years and organ damage using SLICC/ACR-DI was studied.
Results: Out of 100 SLE patients, there were 96 females and 4 males (female to male ratio of 24:1). Mean age of all participants was 30.99 years (range 17-72 years) and average disease duration at the time of enrolment was 3.89 years. Neuropsychiatric manifestations were observed in 84 patients. 20 out of these 84 patients (23.8%) had major neurological manifestations including seizures (13 patients), altered consciousness (10 patients) and cerebro-vascular accident (5 patients). While 7 out of these 84 patients (8.3%) had major psychiatric manifestations including psychosis (5 patients) and depression with suicidal ideation (2 patients). All major neurological manifestations occurred beyond and major psychiatric manifestations occurred within 2 years of diagnosis of SLE. Minor psychiatric manifestations observed included severe anxiety (52 patients), cognitive impairment (43 patients) and mood disorder (25 patients). No statistically significant difference in mean SLICC/ACR-DI score was observed between NPSLE patients and non-NPSLE patients when points received for neurologically related damage were excluded (P=0.10). Neuropsychiatric damage was the most common damage category, followed by renal (25%), pulmonary hypertension (6%), pericarditis (5%), digit loss (4%), venous thrombosis (4%) avascular necrosis (3%), osteomyelitis (2%), muscle weakness (2%) and deforming arthritis (1%).
Conclusions: NPSLE manifestations were seen in 84% of patients, with major neuropsychiatric manifestations in 27% of patients. Headache, seizures and cerebrovascular disease were the most frequent neurological manifestations, while cognitive disorder and severe anxiety being the most common psychiatric manifestations.

Key words: Neuropsychiatric systemic lupus erythematosus, systemic lupus erythematosus, lupus nephritis, lupus cerebritis.