TY - JOUR
T1 - Usefulness of Immunosuppression for Giant Cell Myocarditis
AU - Cooper, Leslie T.
AU - Hare, Joshua M.
AU - Tazelaar, Henry D.
AU - Edwards, William D.
AU - Starling, Randall C.
AU - Deng, Mario C.
AU - Menon, Santosh
AU - Mullen, G. Martin
AU - Jaski, Brian
AU - Bailey, Kent R.
AU - Cunningham, Madeleine W.
AU - Dec, G. William
N1 - Funding Information:
This work was supported by US Food and Drug Administration Grant FD-RO-1-001986 and a Leder Family Philanthropic Grant, Washington, DC. Muromonab-CD3 was supplied by Ortho-Biotek, Inc.
PY - 2008/12/1
Y1 - 2008/12/1
N2 - 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.
AB - 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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U2 - 10.1016/j.amjcard.2008.07.041
DO - 10.1016/j.amjcard.2008.07.041
M3 - Article
C2 - 19026310
AN - SCOPUS:56349160288
SN - 0002-9149
VL - 102
SP - 1535
EP - 1539
JO - American Journal of Cardiology
JF - American Journal of Cardiology
IS - 11
ER -