Fibroelastosis pleuroparenquimatosa e hipertension pulmonar: un fenotipo diferente?
DOI:
https://doi.org/10.53732/rccsalud/2023.e5208Palabras clave:
enfermedades pulmonares intersticiales, fibrosis pulmonar, enfermedades pleurales, fibroelastosis pleuroparenquimatosaResumen
La fibroelastosis pleuroparenquimatosa es una enfermedad pulmonar inusual con características clínicas, radiológicas y patológicas únicas, que se ha incluido recientemente en el consenso actualizado sobre neumonías intersticiales idiopáticas. Su nombre hace referencia a una combinación de fibrosis que involucra la pleura visceral y cambios fibroelastóticos que predominan en el parénquima pulmonar subpleural. Aunque se han descrito varias asociaciones de enfermedades, no se ha identificado de manera inequívoca ninguna causa única. El diagnostico se sustenta en criterios clínicos-radiológicos y de hallazgos histopatológicos cuando se dispone de biopsias. Son escasos los reportes sobre la asociación entre esta entidad y la hipertensión pulmonar. No existe tratamiento hasta la fecha, aunque se relatan sucesos en algunos casos de transplante pulmonar. Describimos las características clínicas de un paciente atendido en un hospital del Paraguay.
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