Giant cell myocarditis: most fatal of autoimmune diseases.
OBJECTIVE
To increase awareness of giant cell myocarditis (GCM), its pathogenesis, and treatment.


METHODS
Review of relevant publications from the English-language literature.


RESULTS
GCM is a rare, frequently fatal inflammatory disorder of cardiac muscle of unknown origin, characterized by widespread degeneration and necrosis of myocardial fibers.Congestive heart failure and ventricular tachycardia are common clinical manifestations. GCM occurs primarily in previously healthy adults, although it is frequently associated with various systemic diseases, primarily of autoimmune causes. The inflammatory infiltrate is characterized by the presence of multinucleated giant cells and is distinct from cardiac sarcoidosis. Animal models of GCM are similar to models of other autoimmune disorders such as rheumatoid arthritis. The prognosis, which is poor despite partial responsiveness to immunosuppressive medications, is improved with cardiac transplantation.


CONCLUSIONS
The clinical and immunopathogenetic similarities with classical rheumatologic diseases, the differential diagnosis with sarcoidosis and other inflammatory conditions, and the use of standard immunosuppressive medications make GCM a disease process that should be added to the rheumatologist's expertise.
Rosenstein
E D
ED
Division of Rheumatology and Arthritis and Rheumatic Disease Center, St. Barnabas Medical Center, Livingston, NJ 07039, USA. Erosenstein@sbhcs.com
Zucker
M J
MJ
Kramer
N
N
D000818:Animals; D001327:Autoimmune Diseases / Q000401:mortality*; D009202:Cardiomyopathies / Q000175:diagnosis; D003937:Diagnosis, Differential; D004195:Disease Models, Animal; D015726:Giant Cells / Q000276:immunology / Q000473:pathology*; D006801:Humans; D009205:Myocarditis / Q000175:diagnosis / Q000209:etiology / Q000276:immunology* / Q000473:pathology* / Q000628:therapy; D012216:Rheumatic Diseases / Q000150:complications; D012507:Sarcoidosis / Q000175:diagnosis