ABSTRACT

Idiopathic pulmonary fibrosis (IPF) is the commonest and one of the deadliest of the interstitial lung diseases (ILDs). As a group, the ILDs are characterized by inflammation and/or fibrosis affecting the space bounded by the alveolar epithelium and the adjacent capillary endothelium. As such, all of the ILDs cause impairment of gas exchange, resulting primarily in the symptom of breathlessness. Of the 200 or more disorders causing ILD, the most frequently encountered of these are the idiopathic interstitial pneumonias (IIPs). This group of disorders, as is discussed in other chapters, subdivide in to a number of separate diseases characterized by differing histopathological appearances (Figure 35.1). IPF represents, when no alternative cause can be identified, the clinical correlate of the histological lesion of usual interstitial pneumonia (UIP).