RESUMEN
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.
Asunto(s)
Enfermedad de Erdheim-Chester/diagnóstico por imagen , Cardiopatías/diagnóstico por imagen , Adulto , Biopsia , Enfermedad de Erdheim-Chester/complicaciones , Enfermedad de Erdheim-Chester/patología , Femenino , Cardiopatías/complicaciones , Cardiopatías/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Tomografía de Emisión de PositronesRESUMEN
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.
Resumo A doença de Erdheim-Chester é uma entidade rara classificada como neoplasia mielóide inflamatória, com incidência desconhecida que ocorre preferencialmente em homens após os 50 anos de idade. Classicamente apresenta-se de forma multissistêmica, sendo sistema esquelético o mais frequentemente comprometido (90% dos pacientes), seguido do sistema geniturinário em 60% dos casos e sistema nervoso central em hipófise e diabetes insipidus em 25% dos casos. As manifestações cardiovasculares estão presentes em mais da metade dos pacientes, sendo a infiltração da aorta e o pseudotumor atrial as formas mais encontradas.