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

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.

journal_name

Arq Bras Cardiol

authors

Costa IBSDS,Abdo ANR,Bittar CS,Fonseca SMR,Moraes ASHT,Kalil Filho R,Pereira J,Hajjar LA

doi

10.5935/abc.20180218

subject

Has Abstract

pub_date

2018-12-01 00:00:00

pages

852-855

issue

6

eissn

0066-782X

issn

1678-4170

pii

S0066-782X2018001800852

journal_volume

111

pub_type

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