%0 Journal Article
%T Idiopathic pulmonary fibrosis
%A Eric B Meltzer
%A Paul W Noble
%J Orphanet Journal of Rare Diseases
%D 2008
%I BioMed Central
%R 10.1186/1750-1172-3-8
%X Idiopathic pulmonary fibrosis (IPF).Synonym: cryptogenic fibrosing alveolitis (CFA) was the preferred term in Europe until terminology was simplified by consensus conference [1].Idiopathic pulmonary fibrosis (IPF) is a chronic disease that manifests over several years and is characterized by scar tissue within the lungs, in the absence of known provocation. Exercise-induced breathlessness and chronic dry cough are the prominent symptoms.IPF belongs to a family of lung disorders known as the interstitial lung diseases (ILD) or, more accurately, the diffuse parenchymal lung diseases (DPLD). Within this broad category of diffuse lung diseases, IPF belongs to the subgroup known as idiopathic interstitial pneumonia (IIP). By definition, the etiology of IIP is unknown. There are seven distinct IIPs, differentiated by specific clinical features and pathological patterns [2]. IPF is the most common form of IIP. It is associated with the pathologic pattern known as usual interstitial pneumonia (UIP); for that reason, IPF is often referred to as IPF/UIP. IPF is usually fatal, with an average survival of approximately three years from the time of diagnosis [3-5]. Older studies suggested that five-year mortality for IPF was only 50%, but this estimate was derived prior to the recognition of nonspecific interstitial pneumonia (NSIP), a pathological subtype of IIP that mimics IPF in its clinical presentation [6-8]. NSIP has a more favorable prognosis and the almost certain inclusion of NSIP cases in older studies of IPF mortality accounts for differences in observed outcome [9]. By definition, IPF/UIP must be discriminated from NSIP.The incidence and prevalence of IPF are difficult to determine because uniform diagnostic criteria have only recently been defined [1]. Historical information relating to vital statistics relied on population studies which utilized diagnostic coding data and death certificates to identify cases. The accuracy of this information can be questioned, espe
%U http://www.ojrd.com/content/3/1/8