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.

摘要 埃尔德海姆-切斯特病（Erdheim-Chester Disease）是一类罕见疾病实体，被归类为炎症性髓系肿瘤，其发病率尚不明确，好发于50岁以上男性。该病典型表现为多系统受累：骨骼系统为最常受累的部位，累及约90%的患者；其次为泌尿生殖系统受累，占比约60%；中枢神经系统受累的病例中约25%累及垂体并伴发尿崩症。超过半数患者存在心血管系统受累表现，其中以主动脉浸润及心房假瘤最为常见。