Chorea in systematic lupus erythematosus; a case report

We report a 33-year-old female who developed a movement disorder during maintenance therapy for systemic lupus erythematosus (SLE). She was diagnosed with SLE at the age of 25, and experienced an episode of SLE-associated hemophagocytic syndrome at age 27, which was successfully treated with intensive immunosuppressive therapy. In November 2012, during maintenance therapy with prednisolone (PSL) 5 mg/day and tacrolimus 0.5 mg/day, she developed acute-onset involuntary movements that were classified as chorea in combination with athetosis in her right limbs and right homonymous hemianopia, which subsided after about 1 h. Her laboratory tests on admission showed an elevated serum anti-double- stranded DNA antibody, positive serum anti-cardiolipin IgG, and an elevated IgG index in cerebrospinal fluid. Magnetic resonance imaging (MRI) showed no significant abnormality on admission, but an ischemic change in her left pallidum appeared on day 7. She was treated with a combination of high-dose corticosteroid, immunosuppressive agents (rituximab, cyclophosphamide, mycofenolate mofetil), antithrombotic therapy (heparin, cilostazol), and dopamine antagonists. Her symptoms remitted partially. Chorea in SLE is recognized as an anti-phospholipid-antibody-associated disorder. In our case, both immunological and ischemic mechanisms were thought to be involved.