EDITORIAL Idiopathic pulmonary fibrosis: a treatable disease?

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease characterised by fibroblast proliferation and extracellular matrix remodelling. The usual treatment strategy is anti-inflammatory, but this is unsuccessful in preventing the fatal outcome in most patients. The disorder is largely unresponsive to corticosteroids and immunosuppressants. Sev-eral papers have recently highlighted the prognostic impor-tance of the new histological classification of the idiopathic interstitial pneumonia, which limits the definition of IPF to the histopathological pattern of usual interstitial pneumonia (UIP) and discriminates several other subgroups with a better prognosis [1–3]. IPF is the most common and most aggressive variant of the chronic idiopathic interstitial pneumonias. One of the hallmarks of IPF/UIP is the formation of multiple patchy fibroblastic foci that evolve to fibrosis, in contrast wit