We here report two patients with steroid-resistant systemic lupus erythematosus (SLE) who were successfully treated with methotrexate (MTX). In both cases, a steroid resistant high fever, associated with mild myositis and pancytopenia were the main common findings, and all these symptoms were alleviated within a few days either by 7.5 mg or 5 mg MTX per week. The number of CD4+ cells increased along with the clinical improvement, whereas the number of CD20+ cells and HLA-DR expressing cells also decreased. Taking into account the side effects of high dose corticosteroids and cyclophosphamides, treatment with a weekly low dose of MTX is known to contribute to an improvement in the long-term prognosis for patients with refractory SLE.