Arq. Bras. Cardiol. 2018; 111(6): 852-855

Cardiovascular Manifestations of Erdheim-Chester’s Disease: A Case Series

Isabela Bispo Santos da Silva Costa, André Neder Ramires Abdo, Cristina Salvadori Bittar, Silvia Moulin Ribeiro Fonseca, Aline Sabrina Holanda Teixeira Moraes, Roberto Kalil Filho, Juliana Pereira, Ludhmila Abrahão Hajjar

DOI: 10.5935/abc.20180218

Abstract

Erdheim-Chester Disease is a rare entity, classified as an inflammatory myeloid neoplasm, with an unknown incidence, occurring preferentially in men after 50 years of age. Classically, it has a multisystemic presentation, with the skeletal system being the most frequently affected (90% of the patients), followed by genitourinary involvement in 60% of cases and central nervous system in the pituitary and diabetes insipidus in 25% of the cases. Cardiovascular manifestations are present in more than half of the patients, with aortic infiltration and atrial pseudotumor being the most common forms.

Cardiovascular Manifestations of Erdheim-Chester’s Disease: A Case Series

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