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Giant cell myocarditis: most fatal of autoimmune diseases
E D Rosenstein1, M J Zucker, N Kramer
1Division of Rheumatology and Arthritis and Rheumatic Disease Center, St. Barnabas Medical Center, Livingston, NJ 07039, USA. Erosenstein@sbhcs.com
Insights
Giant cell myocarditis (GCM) is a rare, fatal heart condition. Early recognition and treatment, including cardiac transplantation, can improve outcomes for this autoimmune-related inflammatory disorder.
Area of Science:
- Cardiology
- Immunology
- Rheumatology
Background:
- Giant cell myocarditis (GCM) is a rare, often fatal inflammatory cardiac muscle disease of unknown etiology.
- It presents with myocardial fiber degeneration and necrosis, leading to heart failure and arrhythmias.
Purpose of the Study:
- To enhance awareness of giant cell myocarditis (GCM).
- To elucidate GCM's pathogenesis and treatment strategies.
Main Methods:
- A comprehensive review of English-language literature was conducted.
- Relevant publications on GCM were analyzed.
Main Results:
- GCM is characterized by multinucleated giant cells, distinct from cardiac sarcoidosis.
- It primarily affects healthy adults, often linked to autoimmune diseases.
- Prognosis is poor but improves with immunosuppression and cardiac transplantation.
Conclusions:
- GCM shares similarities with rheumatologic diseases, necessitating consideration in differential diagnoses.
- Rheumatologists should be familiar with GCM's diagnosis and management.
- Standard immunosuppressive therapies and cardiac transplantation are key treatment modalities.
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.