Fibroproliferation: A New Therapeutic Target for Idiopathic Pulmonary Fibrosis Editorial

Idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) is a progressive fibroproliferative process characterized by the relentless accumulation of extracellular matrix (ECM) in the lung. The outcome is poor and the median survival is 24 to 36 months following diagnosis. Cor-ticosteroids and immunosuppressive drugs, although still recommended, are generally felt to be ineffective in controlling disease progression and have no apparent impact on survival1. Prior reports, citing up to a 20% response rate to these therapies, were uncontrolled and therefore failed to appreciate the biologic variability of the disease in which there can be pro-longed survival (up to 10 years) in 10-20 % of patients. More importantly, previous studies failed to differentiate between IPF/UIP and the other id-iopathic interstitial pneumonias, such as non-specific interstitial pneumo-nia (NSIP) or cryptogenic organizing pneumonia (COP), two disorders known to respond to anti-inflammatory therapy2. The characteristic pathologic feature of IPF/UIP, whether early or lat