Background/Purpose: Sjögren’s disease is recognized as a multifaceted autoimmune disease impacting almost any organ or body system. Of particular importance is the central nervous system (CNS) involvement, which may manifest before classic sicca symptoms. The Sjögren’s Foundation is conducting a systematic review on the treatment of CNS manifestations, the conclusions of which will inform the development of clinical practice guidelines and advance patient outcomes in an often underrecognized aspect of Sjögren’s.

Methods: The Sjögren’s Foundation convened an expert panel to conduct a systematic review as part of a broader CNS guideline project. Our PICO question was: What are effective treatments for Sjögren’s patients >17 years of age with or without an accompanying rheumatic or non-rheumatic autoimmune disorder who are diagnosed with any central nervous system manifestation? Outcomes of interest include mortality, disability, health-related quality of life, any qualitative or quantitative measures of clinically relevant improvement, symptom stabilization, serious adverse events specific to Sjögren’s, activities of daily living, effects on family or caregivers, healthcare resource utilization, and cost-effectiveness. In anticipation of very limited direct evidence in a Sjögren’s population, evidence on treating CNS manifestations in lupus was included as an indirect source of evidence. We searched PubMed, Embase, and ECRI for articles containing prevalence data on 53 CNS manifestations in Sjögren’s disease 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 treatment, published in English). Exclusions were narrative reviews, editorials, case reports, and case series with < 4 cases.

Results: Our initial literature search resulted in 20,549 citations. After duplicates were removed, we screened 17,305. 16,222 articles were excluded based on title and abstract review, and 1083 articles are undergoing full-text review. Due to insufficient data for conducting a meta-analysis, summative narrative results will be presented.

Conclusion: Our preliminary findings underscore both the complexity and the critical importance of accurately identifying and managing CNS manifestations in Sjögren’s disease. Although the initial literature search yielded a high volume of citations, a substantial number did not meet the inclusion criteria, underscoring the relative scarcity of robust data on this topic. Given the limited available evidence specific to Sjögren’s disease, the decision to include parallel data from lupus research provides a necessary indirect comparison to inform clinical decision-making. The results of this systematic review will be essential for developing rigorous, evidence-based recommendations on the evaluation and management of CNS manifestations in Sjögren’s disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/systematic-review-of-therapies-for-central-nervous-system-manifestations-in-sjogrens-disease/