Síndrome coronario agudo y enfermedad de Erdheim-Chester. Patogénesis e implicaciones terapéuticas

Alarcón-García, J.C.; Rodríguez-Suárez, Santiago; García-Ocaña, P; García-Morillo, Salvador

doi:10.1016/j.rccar.2016.06.006

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Diagnosis and Its Difficulties1

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2022

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“…It has already been mentioned that ECD is an entity with very diverse clinical manifestations and that a high clinical suspicion is required for its diagnosis. In our series of patients, the diagnostic delay in the very first patients diagnosed in our unit (patients 1-4) was about 7.5 years; however, in the last patients (4)(5)(6)(7)(8)(9)(10)(11), the diagnostic delay has decreased, and it is frequent that ECD is among the clinical judgments to be discarded in the first visits. At present, the average delay is < 2.5 years.…”

Section: Diagnosis and Its Difficultiesmentioning

confidence: 66%

See 1 more Smart Citation

Diagnosis delay, phenotypic variety, and therapeutic outcome of Erdheim-Chester disease

Trigueros¹,

Lopez-Morales²,

García³

et al. 2022

SJMED

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Erdheim-Chester disease (ECD) is a non-Langerhans histiocytic that typically affects middle-aged adults between the fifth and seventh decades of life. It is characterized by systemic xanthogranulomatous infiltration by histiocytes CD68+/CD1a-. In this paper, we collect the main clinical characteristics of eleven patients, diagnosed with ECD at the Virgen del Rocio Hospital in Seville. After first medical contact, it has been possible to reduce the misdiagnoses and this has shortened the time to diagnosis and initiation of treatment, which has resulted in fewer complications.

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Section: Diagnosis and Its Difficultiesmentioning

confidence: 66%

“…Approximately 75% of patients suffer cardiovascular involvement, conferring a worse prognosis to the disease, being the cause of death in 60% of patients [8][9][10] .…”

Section: Discussionmentioning

confidence: 99%