Cardiac involvement in Erdheim-Chester disease: a case report.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis of unknown etiology characterized by proliferation of lipid-containing foamy histiocytes affecting bones and potentially every organ. There is a wide clinical spectrum of the disease, ranging from asymptomatic bone or soft-tis...

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Detalles Bibliográficos
Autores: Pivatto Júnior, Fernando, Piardi, Diogo Silva, Torres, Felipe Soares
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2015
País:Brasil
Institución:Universidade Federal do Rio Grande do Sul (UFRGS)
Repositorio:Clinical and Biomedical Research
Idioma:inglés
OAI Identifier:oai:seer.ufrgs.br:article/51436
Acceso en línea:https://seer.ufrgs.br/index.php/hcpa/article/view/51436
Access Level:acceso abierto
Palabra clave:Erdheim-Chester disease
heart failure
magnetic resonance imaging
Descripción
Sumario:Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis of unknown etiology characterized by proliferation of lipid-containing foamy histiocytes affecting bones and potentially every organ. There is a wide clinical spectrum of the disease, ranging from asymptomatic bone or soft-tissue lesions to life-threatening systemic involvement. Although the initial descriptions published by Jakob Erdheim and William Chester in 1930 included a patient with right atrial infiltration on autopsy, cardiovascular involvement in ECD has only gained more recognition recently. In the present report, we describe a case with cardiac involvement, presenting with symptomatic heart failure and a cardiac mass assessed with echocardiogram and cardiac magnetic resonance imaging.