Abstract Chorea is a movement disorder observed in 1-2% of systemic lupus erythematosus (SLE) cases. Treatment options include anticonvulsants, dopamine antagonists, antiplatelets, anticoagulants, and immunosuppressants such as cyclophosphamide; however, no specific treatment has been established. Here, we report a 76-year-old woman with chorea associated with antiphospholipid antibodies (aPLs) as well as SLE, who exhibited marked improvement both clinically and serologically following initial treatment with glucocorticoids and subsequent combination therapy with hydroxychloroquine and belimumab. In parallel with the decline in the chorea severity score, all tested aPLs decreased after treatment, with normalization of activated partial thromboplastin time (from 70 to 25 sec), anti-β2-glycoprotein I IgM (from 22 to 12 U/mL), and phosphatidylserine-dependent anti-prothrombin IgG (from 58 to 15 U/mL). A systematic literature review revealed no previously reported cases of chorea associated with SLE treated with a combination of hydroxychloroquine and belimumab. Our case suggests this combination therapy may have a role in maintaining remission and modulating antiphospholipid autoimmunity of SLE-associated chorea.
Asano et al. (2026) studied this question.