Background/Purpose: One common and clinically important form of systemic lupus erythematosus (SLE) is neuropsychiatric systemic lupus erythematosus (NPSLE). This systematic review and meta-analysis aimed to assess the prevalence, regional disparities, and moderators of seven neurological syndromes in adult-onset SLE, as defined by the criteria of the American College of Rheumatology 1999 (ACR1999).

Methods: PubMed, Scopus, Web of Science, and the Cochrane Library were searched for English-language studies (April 1999–April 2024) reporting on CNS neurological syndromes in adults ( >18 years) using the 1999 ACR Case Definitions and including at least 30 patients. Pediatric studies, commentaries, case reports/series, and reviews were excluded. Methodological quality was assessed with the Newcastle-Ottawa Scale. Pooled prevalence with 95% confidence intervals (CIs) was calculated, heterogeneity was evaluated using the I-squared (I²) test, and publication bias was assessed with Egger’s test. Subgroup and meta-regression analyses for moderator and geographic effects were performed using R and a random-effects model.

Results: A total of 25 studies comprising 16,989 patients were included. The mean weighted age was 38.64 years, and 81.1% were female. The median Systemic Lupus International Collaborating Clinics/ACR-damage index (SDI) score at enrollment was 1.1 (range: 0.29–1.7). The most common syndromes were headache (25%, 95% CI [15, 36]), seizures (9%, 95% CI [5, 13]), and cerebrovascular disease (CVD) (9%, 95% CI [5, 13]). In contrast, syndromes such as aseptic meningitis, myelopathy, chorea, and demyelinating syndromes were rare (1%, 95% CI [0, 0.01]). Headache was most prevalent in African populations (55%), followed by Asians (22%), Europeans (19%), and Latin Americans (9%). Seizures were highest among Latin Americans (17%), followed by Asians (13%), Africans (8%), the USA (7%), and Europeans (3%). CVD was most frequent in Asians (13%), then Latin Americans (11%), Europeans (7%), and Africans (3%). Age at SLE diagnosis was not a significant moderator for headache (p=0.59) or CVD (p=0.81), though it showed a trend toward an inverse effect on seizures (p=0.09). Disease duration was not a significant moderator for seizures (p=0.12), but it trended inversely with headache (p=0.07) and CVD (p=0.08). Female gender did not significantly influence headache (p=0.5), seizures (p=0.4), or CVD (p=0.5). Renal involvement was also not a significant moderator for any of the three (headache p=0.76, seizures p=0.87, CVD p=0.81). Substantial heterogeneity and publication bias were present.

Conclusion: Neurological CNS symptoms, as defined per the ACR 1999 criteria, in adult-onset SLE vary significantly. Aseptic meningitis, myelopathy, and chorea were rare, while headaches, seizures, and cerebrovascular disease were most common. Common modifiers like age, sex, disease duration, and renal involvement showed some trends but did not consistently affect syndrome occurrence. Clear regional disparities were noted.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/mapping-neurological-involvement-regional-disparities-and-moderators-of-the-central-nervous-system-in-adult-onset-systemic-lupus-erythematosus-a-global-systematic-review-and-meta-analysis/