Combined immunosuppression for the treatment of idiopathic giant cell myocarditis

Mayo Clin Proc. 1999 Dec;74(12):1221-6. doi: 10.4065/74.12.1221.

Abstract

Giant cell myocarditis (GCM) is a rare and frequently fatal disorder with no proven treatment. Case reports and data from a rat model of GCM suggest that immunosuppressive therapy directed against T lymphocytes may have clinical benefit. We describe a 47-year-old man with severe acute heart failure due to GCM in whom the left ventricular ejection fraction normalized and the myocardial inflammatory infiltrate resolved rapidly after treatment with muromonab-CD3, cyclosporine, azathioprine, and corticosteroids. Three previously published cases with less impressive responses to treatment including muromonab-CD3 and a critical review of the published data on immunosuppressive therapy are included in this report. The response to immunosuppressive therapy is highly variable, and direct comparisons between immunosuppressive regimens do not exist. Therefore, despite individual reports of dramatic improvement after immunosuppressive treatment, firm conclusions cannot be made about the benefit of immunosuppression for GCM. The benefits of immunosuppressive therapy must be confirmed in a prospective, randomized trial.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Anti-Inflammatory Agents / therapeutic use
  • Azathioprine / therapeutic use
  • Cyclosporine / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Giant Cells / pathology*
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Muromonab-CD3 / therapeutic use
  • Myocarditis / drug therapy*
  • Myocarditis / immunology*
  • Myocarditis / pathology
  • Myocarditis / physiopathology
  • Prednisone / therapeutic use
  • Proportional Hazards Models
  • Stroke Volume
  • Treatment Outcome

Substances

  • Anti-Inflammatory Agents
  • Glucocorticoids
  • Immunosuppressive Agents
  • Muromonab-CD3
  • Cyclosporine
  • Azathioprine
  • Prednisone
  • Methylprednisolone