Chorea associated with systemic lupus erythematosus- a systematic review

Objective Chorea is one of the 19 neuropsychiatric manifestations observed in systemic lupus erythematosus (SLE). This article reviews the literature to determine the prevalence and clinical and laboratory features associated with chorea in SLE.Methods We performed a literature review following the PRISMA guidelines and using the following MESH terms in the search and analysis: "Chorea", "movement disorders", "systemic lupus erythematosus", "SLE", "neuropsychiatric manifestations", "neuropsychiatric lupus (NPSLE)", with full articles in English, Portuguese, and Spanish, between September 1999 and January 2024, in PubMed and Scielo.Results 13,922 patients from 60 cohort studies and 41 patients from 18 case/series reports were included. Chorea had a pooled prevalence of 1.1% in SLE patients. We found no association between chorea and clinical or immunological features reported in cohort studies. In case series and case reports, chorea occurred more frequently before or at SLE diagnosis and more frequently in childhood-onset SLE. We propose an investigation and treatment scheme based on the literature review.Conclusion Chorea is a rare manifestation in SLE and factors associated with its occurrence have not been addressed in cohort studies.