Article information
Resumen
Las enfermedades pulmonares intersticiales difusas (EPID) son un grupo heterogéneo de entidades en las que el denominador común es la afectación de la zona anatómica existente entre la membrana basal del epitelio alveolar y el endotelio capilar, conocido como espacio intersticial. El abordaje diagnóstico, las complicaciones que pueden aparecer en la historia natural de estas enfermedades y sus escasas alternativas terapéuticas hacen de ellas un auténtico reto para el clínico. En esta breve revisión se incidirá en los aspectos más relevantes del manejo de las EPID como son los factores pronósticos, las opciones terapéuticas, incluido el papel del trasplante pulmonar, y el diagnóstico y tratamiento de dos complicaciones de trascendental relevancia en su evolución, como son las exacerbaciones y la hipertensión pulmonar asociada.
Palabras clave:
Enfermedad pulmonar intersicial difusa
Exacerbación
Factores pronósticos
Opciones terapéuticas
Abstract
Diffuse interstitial lung disease is a heterogeneous group of diseases in which the common denominator is involvement of the area between the basement membrane of the alveolar epithelium and the capillary endothelium, known as the interstitial space. Diffuse interstitial lung disease poses a tremendous challenge to the clinician due to the diagnostic approach, the complications that can appear in the natural history of these entities, and the scarcity of available therapeutic resources. This brief review discusses key features of the management of diffuse interstitial pulmonary disease, such as prognostic factors, the therapeutic options –including the role of lung transplantation– and the diagnosis and treatment of two complications with crucial impact on the clinical course of the disease: exacerbations and associated pulmonary hypertension.
Keywords:
Diffuse interstitial lung disease
Exacerbation
Prognostic factors
Therapeutic options
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