Via practica 1/2010

Pulmonary fibrosis – diffuse parenchymal lung diseases

Diffuse parenchymal lung diseases (DPLD) comprise more than 200 entities, which include a wide spectrum of conditions, many of unknown aetiology. The idiopathic interstitial pneumonias (IIPs) are a group of diffuse parenchymal lung diseases of unknown etiology with varying degrees of inflammation and fibrosis. Idiopathic pulmonary fibrosis (IPF) is the most common type of IIP associated with a histopathologic pattern of usual interstitial pneumonia (UIP). High-resolution computed tomography and lung function tests are crucial in diagnosis, predicting prognosis and follow up of majority of patients with DPLD. The use of corticosteroids, either alone or with immunosuppressive drugs is usual therapy for patients with DPLD. IPF has the worst prognosis and the poorest response to therapy among the chronic idiopathic interstitial pneumonias.

Keywords: diffuse parenchymal lung diseases, idiopathic pulmonary fibrosis, high-resolution computed tomography, lung function tests, corticosteroids