RESUMEN
Idiopathic pulmonary fibrosis (IPF), a devastating progressive interstitial lung disease (ILD) with no known cause, is the most common and deadly of the idiopathic interstitial pneumonias. With a median survival of 3â»5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. However, in recent years, there has also been progress in the knowledge of genetic factors and biomarkers that may be useful in the prognostic evaluation of these patients. On the other hand, the monitoring of the disease throughout its evolution is key to improving the prognosis of the patients, as it allows for taking therapeutic measures based on this evolution, even early remission for lung transplantation. This article reviews the main prognostic factors of the disease, as well as the most useful way to monitor the disease follow-up.
RESUMEN
Las enfermedades pulmonares intersticiales difusas (EPID) son un grupo heterogéneo de entidades en lasque el denominador común es la afectación de la zona anatómica existente entre la membrana basal delepitelio alveolar y el endotelio capilar, conocido como espacio intersticial. El abordaje diagnóstico, las complicacionesque pueden aparecer en la historia natural de estas enfermedades y sus escasas alternativasterapéuticas hacen de ellas un auténtico reto para el clínico. En esta breve revisión se incidirá en los aspectosmás relevantes del manejo de las EPID como son los factores pronósticos, las opciones terapéuticas, incluidoel papel del trasplante pulmonar, y el diagnóstico y tratamiento de dos complicaciones de trascendentalrelevancia en su evolución, como son las exacerbaciones y la hipertensión pulmonar asociada(AU)
Diffuse interstitial lung disease is a heterogeneous group of diseases in which the common denominator isinvolvement of the area between the basement membrane of the alveolar epithelium and the capillaryendothelium, known as the interstitial space. Diffuse interstitial lung disease poses a tremendous challengeto the clinician due to the diagnostic approach, the complications that can appear in the natural history ofthese entities, and the scarcity of available therapeutic resources. This brief review discusses key featuresof the management of diffuse interstitial pulmonary disease, such as prognostic factors, the therapeuticoptions including the role of lung transplantation and the diagnosis and treatment of two complicationswith crucial impact on the clinical course of the disease: exacerbations and associated pulmonaryhypertension(AU)