Giant cell myocarditis in a 12-year-old girl with common variable immunodeficiency.
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.
Laufs
Helmut
H
Department of Cardiovascular Surgery, The Children's Hospital and Harvard Medical School, Boston, Mass 02115, USA.
Nigrovic
Peter Andrija
PA
Schneider
Lynda C
LC
Oettgen
Hans
H
Del
Nido Pedro
NP
Moskowitz
Ivan P G
IP
Blume
Elizabeth
E
Perez-Atayde
Antonio R
AR
D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000473:pathology; D005260:Female; D015726:Giant Cells / Q000473:pathology; D016027:Heart Transplantation; D006801:Humans; D009205:Myocarditis / Q000150:complications* / Q000473:pathology / Q000601:surgery