Successful combined treatment with thymectomy, rituximab and tocilizumab for severe thymoma-associated multi autoimmune syndrome

Abstract We present a 53-year-old woman who presented simultaneously with acute inflammatory demyelinating polyneuropathy, Graves' disease, leukocytoclastic vasculitis, elevated acetylcholine antibody receptor antibodies and a mediastinal mass. Thymectomy was performed and revealed a type A thymoma and the clinical picture and paraclinical findings were consistent with a thymoma-associated multi-autoimmune syndrome (TAMA). Beside prednisolone and plasmapheresis, the patient was treated with tocilizumab and rituximab. After surgical and immunomodulatory treatment with tocilizumab and rituximab the patient's condition slowly started to improve. TAMA is associated with a spectrum of autoantibodies and immune-mediated damage to multiple organs. Even if thymectomy is crucial for long term prognosis, aggressive immunomodulation should be considered early in the disease course, especially in cases showing involvement of the peripheral and/or central nervous system.