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Objectives 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.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).Abstract PT3:06 Figure 1Conclusions 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. These findings highlight the need for further research to clarify factors influencing CNS involvement in SLE, refine phenotypic classification, and identify specific biomarkers.