Giant cell myocarditis in a 12-year-old girl with common variable immunodeficiency.
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Key Findings
Abstract
Giant cell myocarditis (GCM) is a rare and often fatal disease that infrequently affects children. Common variable immunodeficiency (CVID) describes a heterogeneous group of disorders characterized by hypogammaglobulinemia and poor specific antibody responses. To our knowledge, CVID and GCM have not been reported together in 1 patient. We describe a 12-year-old girl with CVID who developed acute severe GCM that necessitated cardiac transplantation. Histopathological and immunohistochemical studies of the endomyocardial biopsy specimen and the explanted heart revealed numerous histiocytes, eosinophils, T cells, and multinucleated giant cells. Both CVID and GCM are thought to involve dysregulation of T-cell function and have been associated with a similar spectrum of autoimmune conditions. The coincidence of CVID and GCM in a single patient may reflect a pathophysiologic connection.
Affiliation
Department of Cardiovascular Surgery, The Children's Hospital and Harvard Medical School, Boston, Mass 02115, USA.
External References
- PubMed ID:
- 11795251
- DOI:
- 10.4065/77.1.92
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