Fase acelerada de la fibrosis pulmonar idiopática / Accelerated phase of idiopathic pulmonary fibrosis

RESUMEN

La historia natural de la fibrosis pulmonar idiopática es la lenta evolución hacia la insuficiencia respiratoria y la muerte. Sin embargo, un pequeño porcentaje de casos evoluciona en semanas o pocos meses de forma fulminante con insuficiencia respiratoria, imágenes extensas de vidrio esmerilado y formación de membranas hialinas en las muestras de biopsia; es lo que se denomina "fase acelerada" de la fibrosis pulmonar idiopática, durante la cual se describen incrementos de las cifras de reactantes de fase aguda y marcadores tumorales. Hasta la fecha no se ha relacionado el marcador tumoral monoclonal CA 15/3 con dicha fase acelerada

ABSTRACT

The natural history of idiopathic pulmonary fibrosis is characterized by a slow progression resulting in respiratory failure and death. The progression to the fulminant form is rapid in a small percentage of cases, however. Within weeks or months, patients develop respiratory distress, and extensive ground-glass patterns can be seen in computed tomography scans and hyaline membranes in biopsy samples. This is described as an accelerated phase of idiopathic pulmonary fibrosis, in which elevated levels of acute-phase reactants and tumor markers have been reported. To date, the monoclonal tumor marker, CA 15/3 has not been associated with the accelerated phase