Parallel improvement of chorea and antiphospholipid autoimmunity during combination therapy with hydroxychloroquine, belimumab, and glucocorticoids in systemic lupus erythematosus

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 the case of a 76-year-old woman with chorea associated with antiphospholipid antibodies 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 antiphospholipid antibodies decreased after treatment, with the normalisation of activated partial thromboplastin time (from 70 to 25 s), anti-β2-glycoprotein I IgM (from 22 to 12 U/ml), and phosphatidylserine-dependent antiprothrombin 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 that this combination therapy may have a role in maintaining remission and modulating the antiphospholipid autoimmunity of SLE-associated chorea.