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

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

Arq Bras Cardiol. 2018 Dec;111(6):852-855. doi: 10.5935/abc.20180218.

[Article in English, Portuguese]

Authors

Isabela Bispo Santos da Silva Costa¹, André Neder Ramires Abdo¹, Cristina Salvadori Bittar¹, Silvia Moulin Ribeiro Fonseca¹, Aline Sabrina Holanda Teixeira Moraes¹, Roberto Kalil Filho², Juliana Pereira¹, Ludhmila Abrahão Hajjar^{1

2}

Affiliations

¹ Instituto do Câncer do Estado de São Paulo, São Paulo, SP - Brazil.
² Instituto do Coração (InCor) - Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP - Brazil.

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.

Publication types

Case Reports

MeSH terms

Adult
Biopsy
Erdheim-Chester Disease / complications
Erdheim-Chester Disease / diagnostic imaging*
Erdheim-Chester Disease / pathology
Female
Heart Diseases / complications
Heart Diseases / diagnostic imaging*
Heart Diseases / pathology
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Positron-Emission Tomography