Background/Purpose: Sjögren’s disease is a systemic autoimmune disorder with diverse clinical manifestations affecting multiple organ systems. While dry mouth, dry eyes, fatigue and pain are the most common features, involvement of the central nervous system (CNS) is increasingly reported among Sjögren’s patients. CNS manifestations may precede Sjögren’s diagnosis and can occur in the absence of classic sicca symptoms, potentially leading to delayed or missed diagnoses. To better understand the neurological aspects, we conducted a scoping review and meta-analysis to characterize the prevalence of CNS manifestations in Sjögren’s disease.

Methods: The Sjögren’s Foundation convened an expert panel to conduct this scoping review as part of a broader CNS guideline project. We searched PubMed, Embase, and ECRI for articles containing prevalence data on CNS manifestations in Sjögren’s from 1980 to September 16, 2024. All articles were screened in duplicate by members of the panel using predefined inclusion criteria ( >17 years of age; any sex, gender, ethnicity, or location; any study design; published in English). Studies that were not conducted in a general Sjögren’s population were excluded. One author (JFH) extracted data on event counts and total sample size, and a second author (AV) conducted an audit of the extracted data. We used the meta package in R to conduct random-effects meta-analyses of proportions. To stabilize the variance of proportions, we used the Freeman-Tukey double arcsine transformation. We estimated between-study heterogeneity using the restricted maximum likelihood (REML) method.

Results: Our literature search resulted in 20,549 citations. After duplicates were removed, we screened 17,305. 110 articles met the inclusion criteria. Prevalence data for the following CNS manifestations were identified: anxiety, aseptic meningitis, bipolar, brain stem-related symptoms, central sensitization, cerebellar syndrome, cerebral vasculitis, cerebral vascular disease, chronic fatigue syndrome, cognitive dysfunction/impairment, dementia, demyelinating diseases, depression, encephalopathy/delirium, fibromyalgia, headache, insomnia, long-COVID-CNS manifestations, migraine, movement disorders, multiple sclerosis, myelopathy, neuromyelitis optica spectrum disorder, optic neuritis, Parkinson’s disease, psychosis, restless leg syndrome, schizophrenia, seizure disorder, stroke, transverse myelitis. Meta-analysis of proportions for each of these CNS manifestations will be presented.

Conclusion: This scoping review and meta-analysis of proportions highlights the prevalence of CNS manifestations in individuals with Sjögren’s. By systematically identifying and quantifying these neurological features, this study provides critical insights to inform earlier recognition, prompt referral, and improved multidisciplinary management of Sjögren’s disease. These results also support the need for future guidelines and research focused specifically on CNS manifestations to enhance care for this complex patient population.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prevalence-of-central-nervous-system-manifestations-in-sjogrens-disease/