Idiopathic pulmonary fibrosis (IPF) is the most common of the interstitial pneumonias. IPF is confirmed by the identification of usual interstitial pneumonia (UIP) on surgical lung biopsy. The idiopathic interstitial pneumonias (IIPs) are a heterogeneous group of non neoplastic disorders resulting from damage to the lung parenchyma by varying patterns of inflammation and fibrosis. The IIPs include the entities of IPF, non-specific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), acute interstitial pneumonia (AIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP) and lymphocytic interstitial pneumonia (LIP). It is important to emphasize that IPF is a progressive and irreversible illness. Until now, there has been no available drug that has been able to modify the progressive natural course of IPF and its usual terminal outcome. It is characterized by radiographically evident interstitial infiltrates predominantly affecting the lung bases, by progressive dyspnea, by worsening of pulmonary function with restrictive impairment, pathologically by fibroblast proliferation and exracellular matrix accumulation resulting in irreversible distortion of the architecture of the lung.
|Translated title of the contribution||Idiopathic pulmonary fibrosis|
|Number of pages||18|
|Journal||Rassegna di Patologia dell'Apparato Respiratorio|
|Publication status||Published - Apr 2006|
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Pulmonary and Respiratory Medicine