NEUROPSYCHIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS CLINICAL FEATURES AND ORGAN DAMAGE IN A SINGLE RHEUMATOLOGY CENTER IN MALAYSIA

Abstract

Background: Neuropsychiatric systemic lupus erythematosus (NPSLE) poses a significant challenge in diagnosis and management, and is associated with increased morbidity and mortality, despite advances in systemic lupus erythematosus (SLE) management.

Objectives: We aimed to examine the prevalence, clinical features, and the organ damage of NPSLE patients in our SLE cohort.

Methods: We collected demographics and clinical data in this prospective observational study from July 2018 until November 2025. All patients fulfilled the 2012 Systemic Lupus Collaborating Clinics (SLICC) Classification Criteria for SLE. NPSLE cases were defined according to the 1999 ACR nomenclature and case definitions for NPSLE. Statistical analysis used independent sample T-test for continuous data and Chi-Square or Fisher’s exact test for categorical data. A p-value<0.05 is considered significant.

Results: There were 748 SLE patients in this cohort, with 663 (88.6%) women and 85(11.4%) men. The mean age at diagnosis was 31.14 ±13.85 years. There were 377 (50.4%) patients with lupus nephritis. There were 71 (9.5%) patients with neuropsychiatric lupus (NPSLE) manifestations. The majority of NPSLE patients had seizures (n=32,45.1%), followed by cerebrovascular disease (n=9,12.7%), acute confusional state (n=6,8.5%), myelopathy (n=4,5.6%), and mixed presentation (n=4,5.6%). Other NPSLE presentations were aseptic meningitis (n=3,4.2%), psychosis (n=3,4.2%), mononeuropathy (n=3,4.2%), cranial neuropathy (n=3,4.2%), polyneuropathy (n=2,2.8%), cognitive dysfunction (n=1,1.4%) and mood disorder (n=1,1.4%). In this NPSLE cohort, 45 (63.4%) patients were positive for anti-dsDNA, 11(15.5%) were positive for antiphospholipid antibodies, and 8(11.3%) were positive for anti-Ribosomal P protein. More patients in the NPSLE group had severe disease at first presentation compared to the non-NPSLE group (51(71.8%) vs 293(43.3%), p<0.001). A higher proportion of patients in the NPSLE group had a SLE Damage Index score >1 compared with the non-NPSLE group (26(36.6%) vs 103(15.2%), p<0.001). The mean SLE Damage Index score was also higher in the NPSLE group (1.38±1.52 vs 0.61±1.10, p<0.001). Certain SLE Damage Index score items were also significantly higher in the NPSLE group as compared to the non-NPSLE group. The items were retinal change (3(4.2%) vs 6(0.89%), p=0.05), seizures (13(18.3%) vs 5(0.74%), p<0.001), stroke (14(19.7%) vs 21(3.1%),p<0.001), cranial or peripheral neuropathy (3(4.2%) vs 2(0.3%), p=0.007), osteoporosis (6(8.5%) vs 22(3.2%), p=0.04), and chronic scarring alopecia (5(7.04%) vs 9(1.33%), p=0.007). There were more deaths in the NPSLE group compared with the non-NPSLE group (23.9% vs 11.8%, p=0.008).

Conclusions: The most common NPSLE manifestation was seizure. Although not as prevalent as lupus nephritis, NPSLE still carried significant morbidity and mortality, with NPSLE associated with severe SLE manifestation at diagnosis, neurology organ damage items and death.