Total lymphoid irradiation: new therapeutic option for refractory giant cell myocarditis

Idiopathic giant cell myocarditis (GCM) is believed to be a T-lymphocyte-mediated autoimmune disease. Some patients with GCM have a dramatic clinical response to anti-T-cell immunosuppression. However, this response is not uniform and patients often deteriorate rapidly and need a cardiac transplantation within months of diagnosis. Following cardiac transplantation, GCM may recur in the graft but is usually mild and responds to augmentation of immunosuppression. This report is the first description of total lymphoid irradiation (TLI) for the treatment of GCM, which was used in a patient who developed an exceptionally early and severe recurrence of GCM in the cardiac graft that remained refractory to heightened immunosuppression for 4 months. Clinical and histologic remission followed a course of TLI and was maintained for 1 year despite a gradual decrease in immunosuppression. This novel treatment should be considered in all patients with GCM who do not have histologic remission with the currently employed anti-T-cell immunosuppression.