New perspectives in the treatment of idiopathic pulmonary fibrosis.
Idiopathic pulmonary fibrosis (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 fibrotic lung disorder leading to death within a median duration of 3 years. It was hypothesized in the 1970s that pulmonary fibrosis initiates as an "alveolitis" progressing to interstitial fibrosis 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 fibrotic process in the absence of macrophages and with minimal inflammation. 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-fibrotic or profibrotic mediators, oxidant injury, exaggerated coagulation, thus leading to investigate new treatment strategies. Preliminary results of some of such trials have shown significant reductions in lung function decline, disease exacerbation and mortality.
Rogliani
Paola
P
Policlinico Universitario Tor Vergata, Viale Oxford 81, 00133 Rome, Italy. paola.rogliani@uniroma2.it.
Mura
Marco
M
Assunta Porretta
Maria
M
Saltini
Cesare
C
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D000975:Antioxidants / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D004848:Epithelium / Q000503:physiopathology; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007249:Inflammation / Q000503:physiopathology; D007371:Interferon-gamma / Q000627:therapeutic use; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
D000305:Adrenal Cortex Hormones; D020533:Angiogenesis Inhibitors; D000975:Antioxidants; D000998:Antiviral Agents; D007166:Immunosuppressive Agents; D010726:Phosphodiesterase Inhibitors; D014409:Tumor Necrosis Factor-alpha; D007371:Interferon-gamma