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Definición, patogenia y factores de riesgo de la fibrosis pulmonar idiopática / Definition, pathogenesis and risk factors of idiopathic pulmonary fibrosis
Reyes C, Felipe.
  • Reyes C, Felipe; SER Chile. Comisión de Enfermedades Pulmonares Intersticiales Difusas. Santiago. CL
Rev. chil. enferm. respir ; 35(4): 261-263, dic. 2019. graf
Article in Spanish | LILACS | ID: biblio-1092702
RESUMEN
La fibrosis pulmonar idiopática (FPI) es una forma específica de neumonía intersticial idiopática, de tipo fibrosante crónica y progresiva, con patrón radiológico y/o histológico de neumonía intersticial usual (NIU). Su patogenia es compleja, el modelo más aceptado actualmente es basado en las células epiteliales alveolares, aberrantemente activadas que conducen a la proliferación de fibroblastos y su diferenciación a miofibroblastos que depositan matriz extracelular y destruyen irreversiblemente la arquitectura pulmonar. No existe un claro factor inicial que explique la activación y posterior mantención del mecanismo de la fibrosis. El factor de crecimiento transformante beta (TGF-β) liberado por las células epiteliales alveolares se ha implicado como unos de los principales conductores de la inducción y proliferación de fibroblastos alterados que persiste mucho tiempo después de la estimulación inicial, lo que explicaría en gran parte el comportamiento clínico progresivo y crónico.
ABSTRACT
Idiopathic pulmonary fibrosis (IPF) is a specific form of idiopathic interstitial pneumonia, of chronic and progressive fibrosing type, with radiological and / or histological pattern of usual interstitial pneumonia (UIP). Its pathogenesis is complex, the most accepted model currently is based on the fact that the alveolar epithelial cells, aberrantly activated, lead to the proliferation of fibroblasts and their differentiation to myofibroblasts that deposit extracellular matrix and irreversibly destroy the pulmonary architecture. There is no clear initial trigger that explains the activation and subsequent maintenance of the fibrosis mechanism. The transforming growth factor beta (TGF-β), released by the alveolar epithelial cells, has been implicated as one of the main drivers of the induction and proliferation of altered fibroblasts that persists long after the initial stimulation, which would largely explain progressive and chronic clinical behavior.
Subject(s)


Full text: Available Index: LILACS (Americas) Main subject: Idiopathic Pulmonary Fibrosis Type of study: Etiology study / Practice guideline / Prognostic study / Risk factors Limits: Humans Language: Spanish Journal: Rev. chil. enferm. respir Journal subject: Pulmonary Disease (Specialty) Year: 2019 Type: Article Affiliation country: Chile Institution/Affiliation country: SER Chile/CL

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Full text: Available Index: LILACS (Americas) Main subject: Idiopathic Pulmonary Fibrosis Type of study: Etiology study / Practice guideline / Prognostic study / Risk factors Limits: Humans Language: Spanish Journal: Rev. chil. enferm. respir Journal subject: Pulmonary Disease (Specialty) Year: 2019 Type: Article Affiliation country: Chile Institution/Affiliation country: SER Chile/CL