Refractory Thymoma associated Myasthenia Gravis Successfully Treated with Cyclical Intravenous Immunoglobulins: Case-Based Review

Abstract

Myasthenia gravis (MG) is an autoimmune neuromuscular junction disease characterized by easy fatigability of skeletal muscles. Thymoma associated myasthenia gravis (T-MG) is defined as MG occurring with a concurrent thymoma. T-MG is associated with more severe generalised and bulbar disease. T-MG is also a risk factor for developing MG refractory to standard immunosuppression therapy. We report a case of T-MG with multiple relapses despite undergoing a thymectomy. Her relapses were also refractory to escalated immunosuppressive therapy (azathioprine and mycophenolate mofetil). Her disease dramatically stabilised on cyclical intravenous immunoglobulin therapy combined with maintenance oral corticosteroids. We also discuss newer therapeutic options for refractory myasthenia gravis including monoclonal antibodies against C20 (rituximab) and C5 complement component (eculizumab).