Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis.

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 3⁻5 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.

[Key features in the management of diffuse interstitial pulmonary disease].

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.