title: Giant cell myocarditis in a 12-year-old girl with common variable immunodeficiency.
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.
authors.affiliation: Department of Cardiovascular Surgery, The Children's Hospital and Harvard Medical School, Boston, Mass 02115, USA.
authors.forename: Helmut
authors.initials: H
authors.lastname: Laufs
authors.forename: Peter Andrija
authors.initials: PA
authors.lastname: Nigrovic
authors.forename: Lynda C
authors.initials: LC
authors.lastname: Schneider
authors.forename: Hans
authors.initials: H
authors.lastname: Oettgen
authors.forename: Nido Pedro
authors.initials: NP
authors.lastname: Del
authors.forename: Ivan P G
authors.initials: IP
authors.lastname: Moskowitz
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Blume
authors.forename: Antonio R
authors.initials: AR
authors.lastname: Perez-Atayde
mesh_terms: 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