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Review: New perspectives in the treatment of idiopathic pulmonary fibrosis

Policlinico Universitario Tor Vergata, Viale Oxford 81, 00133 Rome, Italy, saltini{at}med.uniroma2.it, paola.rogliani{at}uniroma2.it

Marco Mura

UOC Malattie Respiratorie, Policlinico Tor Vergata, University of Rome "Tor Vergata

Maria Assunta Porretta

UOC Malattie Respiratorie, Policlinico Tor Vergata, University of Rome "Tor Vergata

Cesare Saltini

Policlinico Universitario Tor Vergata, Viale Oxford 81, 00133 Rome, Italy

Idiopathic pulmonary "brosis (IPF) is the most frequent idiopathic interstitial pneumonia with a prevalence ranging from 5 to 15 per 100,000 persons, and above 175 per 100,000 in the older population. IPF is a relentlessly progressive "brotic lung disorder leading to death within a median duration of 3 years.

It was hypothesized in the 1970s that pulmonary "brosis initiates as an "alveolitis" progressing to interstitial "brosis with connective tissue deposition, derangement of the lung architecture and functional impairment.

However, in vitro studies indicated that alveolar/bronchiolar injured epithelial cells can drive the "brotic process in the absence of macrophages and with minimal in"ammation. This, together with the inability of classic immunosuppressive therapy to cure IPF, generated new pathogenesis paradigms and intense research into the role of the lack or the excessive production of anti-"brotic or pro"brotic mediators, oxidant injury, exaggerated coagulation, thus leading to investigate new treatment strategies. Preliminary results of some of such trials have shown signi"cant reductions in lung function decline, disease exacerbation and mortality.

Key Words: idiopathic pulmonary "brosis • idiopathic interstitial pneumonia • treatment

Therapeutic Advances in Respiratory Disease, Vol. 2, No. 2, 75-93 (2008)
DOI: 10.1177/1753465808089363


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