Disease Information for Pulmonary fibrosis, Primary/H-R disease: Definition

  • Pneumonia, Interstitial;Bronchiolitis Obliterans Organizing Pneumonia (Boop);Chronic Fibrous Pneumonia; Diffuse Alveolar Damage; Fibrous Interstitial Pneumonia; Giant Cell Interstitial Pneumonia; Idiopathic Interstitial Pneumonia;

    Usual Interstitial Pneumonia (UIP); Subdivisions: Acute Interstitial Pneumonia (AIP); Cryptogenic Organizing Pneumonia (COP); Desquamative Interstitial Pneumonia (DIP);

    Idiopathic Pulmonary Fibrosis (IPF); Lymphoid Interstitial Pneumonia (LIP); Nonspecific Interstitial Pneumonia (NSIP);

    Respiratory Bronchiolitis-Associated Interstitial Lung Disease (RB-ILD); The abnormal accumulation of inflammatory cells in lung tissue may lead to any one of several disorders with similar signs and symptoms; As white blood cells and protein-rich plasma build up in the air sacs of the lungs (alveoli), inflammation is generated; The inflammatory process, if it lasts long enough, may harden the fluid and the resultant firm, fibrous substance (scarring) may replace the lung tissue; If the scarring is extensive, the air sacs may be destroyed over time and the resultant space replaced by cysts; The American Thoracic Society and the European Respiratory Society jointly studied the interstitial pneumonias during 2001 and issued a Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias; This consensus statement was intended to replace several older classification schemes that had led to a confusion of names and syndromes; Participants agreed that the Idiopathic Interstitial Pneumonias (IIPs) comprise a number of clinical entities that are each rare and sufficiently different from one another to be considered as distinct disorders; This report follows the joint ARS/ERS classification-------------[NORD 2005]-------