A new study has found that patients with neuropsychiatric systemic lupus erythematosus have 3 times increased risk of death, and this risk increases to over 7 times higher in patients with focal CNS symptoms.
A new study has found that individuals with neuropsychiatric systemic lupus erythematosus (SLE, or lupus) have 3 times increased risk of death, and this risk increases to over 7 times higher in patients with focal central nervous system (CNS) symptoms.
Individuals with lupus can experience a wide range of neuropsychiatric problems. These range from mild to moderate, and can include headache, seizures, autonomic disorders, demyelinating syndromes, and psychosis, to name a few. The presence of neuropsychiatric disorders often indicates a worse prognosis. Yet research in this area is limited. Studies have about risk factors for the development of neuropsychiatric lupus have generally been small and results have been inconsistent.
Researchers conducted a prospective observational study between 1998 and 2015. The study, led by Sang-Cheol Bae, MD, PhD, of Hanyang University Hospital for Rheumatic Diseases (Seoul, South Korea), included 1121 patients with lupus. Of these, neuropsychiatric symptoms were found in 429 patients as defined by American College of Rheumatology (ACR) criteria and 216 as defined by Ainiala criteria. The Ainiala criteria have a narrower definition of neuropsychiatric lupus than the ACR criteria, and may have less likelihood of including neuropsychiatric symptoms that are not actually caused by lupus.
At baseline and annually, researchers assessed autoantibodies, as well as disease activity using Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and disease damage using the Systemic Lupus International Collaborating Clinic (SLICC)/ACR Damage Index.
Results were adjusted for age at diagnosis, disease activity at diagnosis, disease damage at diagnosis, hypertension, year of education, and number of fulfilled ACR criteria at diagnosis.
Three times increased risk of mortality with neuropsychiatric lupus (HR 3.09, CI 1.03–9.21, p=0.04)
Over 7 times increased risk of death with focal CNS symptoms (HR=7.83, CI 2.12–28.96, p<0.01)
Increased odds of neuropsychiatric lupus linked to:
Higher SLEDAI score at diagnosis (OR 1.08, CI 1.01–1.16, p=0.02)
Antiphospholipid antibody positivity (OR 1.72, CI 1.03–2.87, p=0.04)
Decreased odds of neuropsychiatric lupus linked to:
Elevated anti-dsDNA antibodies (OR 0.43, CI 0.24–0.78, p<0.01)
More years of education (OR 0.92, CI 0.85–1.00, p=0.04)
Stroke and seizure constituted the most frequent combination of symptoms. This combination occurred in 23.4% of patients with stroke, of which 14.9% had early seizures within 14 days of stroke. This percentage is higher than post-stroke seizure reported in the general population (between 5% to 20% overall, and between 3% to 7% for early post-stroke seizure), according to studies quoted in the article.
The study is the first to report that the risk of post-stroke seizure might be higher in SLE patients with neuropsychiatric lupus compared to the general population. Further study is needed about individual symptoms, as well as the clinical significance of symptom combinations. The study was conducted in a single tertiary referral center in Korea, and results may not apply to all individuals with lupus.
• Single center prospective study in Korea found that individuals with neuropsychiatric lupus have 3 times increased risk of death, and those with focal CNS symptoms have over 7 times increased risk of death
• Increased odds of neuropsychiatric lupus were linked to higher disease activity at diagnosis and antiphospholipid antibody positivity
• Decreased odds of neuropsychiatric lupus were linked to anti-dsDNA antibody positivity and more years of education
• Individuals with lupus may be at higher risk for post-stroke seizure; further research is needed on this issue
1. Ahn GY, Kim D, Won S, et al. Prevalence, risk factors, and impact on mortality of neuropsychiatric lupus: a prospective, single-center study. Lupus. 2018 Jan 1:961203318772021. doi: 10.1177/0961203318772021