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.
Cooper
Leslie T
LT
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA. cooper.leslie@mayo.edu
Hare
Joshua M
JM
Tazelaar
Henry D
HD
Edwards
William D
WD
Starling
Randall C
RC
Deng
Mario C
MC
Menon
Santosh
S
Mullen
G Martin
GM
Jaski
Brian
B
Bailey
Kent R
KR
Cunningham
Madeleine W
MW
Dec
G William
GW
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001379:Azathioprine / Q000627:therapeutic use; D016572:Cyclosporine / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D015726:Giant Cells / Q000473:pathology*; D006801:Humans; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D016853:Muromonab-CD3 / Q000008:administration & dosage / Q000627:therapeutic use*; D009205:Myocarditis / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D011446:Prospective Studies; D012042:Registries; D028761:Withholding Treatment; D055815:Young Adult
D007166:Immunosuppressive Agents; D016853:Muromonab-CD3; D016572:Cyclosporine; D001379:Azathioprine