A 42-year-old woman was admitted to our hospital because of skin ulcers and an arthritis. She had been suffered from rheumatoid arthritis (RA) since she was 18 years old. Disease activity of RA increased in October, 2001. Neuropsychiatric symptoms such as mood fluctuations and cognitive deficit presented and deteriorated in June, 2002. Based on clinical features and immunologic tests, she was diagnosed with overlap syndrome of RA and systemic lupus erythematosus (SLE) with various clinical conditions such as hemophagocytic syndrome, skin ulcersdue to vasculitis, and CNS lupus. The disease activity of SLE decreased and confusion diminished with the application of the steroid therapy, but schizophreniform disorder, cognitive dysfunction, memory disturbance, and cognitive deficit did not improve. The treatment was changed to a combination therapy of steroid, azathioprine, immune adsorption, and intrathecal injection with methotrexate plus dexamethasone. CNS lupus was markedly improved by these combination therapies. We also demonstrated the utility of CNS lupus markers such as IL-6, anti-neuronal cell antibody, anti-ribosomal P antibody in serum and cerebrospinal fluid. Anti-neuronal cell antibody in cerebrospinal fluid was useful in this case. There have been a few reports of intrathecal methotrexate plus dexamethasone injection therapy for CNS lupus. Based on the information obtained in this case, we discussed the efficacy of immunosuppressive therapies including intrathecal injection and the method for evaluating disease activity in CNS lupus.
