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Diffuse interstitial lung diseases form a group of respiratory diseases about which many questions remain to be answered. In recent years there have been major advances in the correct diagnostic classification of each disease, and therefore, the essential foundations have been laid for investigation of their pathophysiology. However, both the triggers and the precise mechanisms that lead to irreversible changes in the lung parenchyma remain to be identified. Idiopathic pulmonary fibrosis is the most common diffuse interstitial lung disease and has the worst prognosis. Current treatments are empirical and the response is random; furthermore, they do not improve survival. Consequently, most basic research has focused on the pathophysiology of the disease and on identifying an effective therapeutic approach. The aim of this review is to describe the experimental studies that have begun to open the way towards an understanding of the complex process of fibrosis.
Key words:
Pulmonary fibrosis
Experimental studies
Interstitial lung disease
Las enfermedades pulmonares intersticiales difusas son un grupo de enfermedades respiratorias con múltiples incógnitas por resolver. En los últimos años se ha asistido a un gran avance en la clasificación para el diagnóstico correcto de cada una de ellas, con lo que se han sentado las bases indispensables para el estudio del proceso fisiopatológico en cada entidad. Sin embargo, resultan desconocidos tanto la causa desencadenante como los mecanismos exactos que llevan a la alteración irreversible del parénquima. Dentro de las enfermedades pulmonares intersticiales difusas, la más frecuente y de peor pronóstico es la fibrosis pulmonar idiopática. Los tratamientos actuales son empíricos, con respuesta aleatoria, e incapaces de mejorar la supervivencia. Por este motivo la mayoría de los estudios básicos se han centrado en buscar respuestas sobre su fisiopatología y un abordaje terapéutico efectivo. El objetivo de esta revisión es dar a conocer los estudios experimentales que han empezado a abrir caminos hacia la comprensión del complejo proceso fibrótico.
Palabras clave:
Fibrosis pulmonar
Estudio experimental
Enfermedad pulmonar intersticial
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