Rituximab as an Effective Treatment for New-onset Evans Syndrome and Systemic Lupus Erythematosus with Lupus Nephritis

Evans syndrome (ES) is characterized by the development of autoimmune hemolytic anemia and immune thrombocytopenic purpura and is often linked to autoimmune diseases, such as systemic lupus erythematosus (SLE). Standard treatment includes prednisolone and intravenous immunoglobulin; however, relapse commonly occurs when prednisolone is tapered or stopped. Rituximab is increasingly used for refractory ES with SLE, although its efficacy in new-onset cases remains unclear. We herein report a 67-year-old woman with new-onset ES and SLE with lupus nephritis class IV-G whose condition improved with rituximab after prednisolone, hydroxychloroquine, and mycophenolate mofetil. The patient remained relapse-free for one year, suggesting that rituximab is a potentially viable first-line therapy.