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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Usefulness of immunosuppression for giant cell myocarditis.

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.[1]


  1. Usefulness of immunosuppression for giant cell myocarditis. Cooper, L.T., Hare, J.M., Tazelaar, H.D., Edwards, W.D., Starling, R.C., Deng, M.C., Menon, S., Mullen, G.M., Jaski, B., Bailey, K.R., Cunningham, M.W., Dec, G.W. Am. J. Cardiol. (2008) [Pubmed]
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