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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
Usefulness of immunosuppression for giant cell myocarditis
Leslie T Cooper1, Joshua M Hare, Henry D Tazelaar
1Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA. cooper.leslie@mayo.edu
Insights
Giant cell myocarditis (GCM) treatment with immunosuppression improves survival. However, stopping immunosuppression risks fatal GCM recurrence, highlighting the need for careful management of this rare heart condition.
Area of Science:
- Cardiology
- Immunology
- Pathology
Background:
- Giant cell myocarditis (GCM) is a rare, lethal cardiac disorder.
- Previous studies lacked detailed cardiac function assessments and prospective data.
- Limited understanding of immunosuppression efficacy and recurrence risks exists.
Purpose of the Study:
- To prospectively evaluate immunosuppression for GCM.
- To assess treatment impact on cardiac pathology and survival.
- To investigate the risk of GCM recurrence after immunosuppression withdrawal.
Main Methods:
- Prospective, multicenter study of GCM patients (n=20).
- Treatment included high-dose steroids, cyclosporine, and muromonab-CD3.
- Serial endomyocardial biopsies assessed treatment response.
Main Results:
- Immunosuppression decreased necrosis, inflammation, and giant cells (p=0.001).
- One-year survival was high, with 2 heart transplants and 1 death from unrelated complications.
- A fatal GCM recurrence occurred after immunosuppression withdrawal.
Conclusions:
- Prospective immunosuppression therapy improves long-term survival in GCM.
- Withdrawal of immunosuppression carries a risk of fatal GCM recurrence.
- Careful management and long-term monitoring are crucial for GCM patients.
Abstract:
Giant cell myocarditis (GCM) is a rare and highly lethal disorder. The only multicenter case series with treatment data lacked cardiac function assessments and had a retrospective design. We conducted a prospective, multicenter study of immunosuppression including cyclosporine and steroids for acute, microscopically-confirmed GCM. From June 1999 to June 2005 in a standard protocol, 11 subjects received high dose steroids and cyclosporine, and 9 subjects received muromonab-CD3. In these, 7 of 11 were women, the mean age was 60 +/- 15 years, and the mean time from symptom onset to presentation was 27 +/- 33 days. During 1 year of treatment, 1 subject died of respiratory complications on day 178, and 2 subjects received heart transplantations on days 2 and 27, respectively. Serial endomyocardial biopsies revealed that after 4 weeks of treatment the degree of necrosis, cellular inflammation, and giant cells decreased (p = 0.001). One patient who completed the trial subsequently died of a fatal GCM recurrence after withdrawal of immunosuppression. Her case demonstrates for the first time that there is a risk of recurrent, sometimes fatal, GCM after cessation of immunosuppression. In conclusion, this prospective study of immunosuppression for GCM confirms retrospective case reports that such therapy improves long-term survival. Additionally, withdrawal of immunosuppression can be associated with fatal GCM recurrence.
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