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

Arq Bras Cardiol; 111(6): 852-855, 2018 Dec.
Artigo em Inglês, Português | MEDLINE | ID: mdl-30517380
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.