TY - JOUR
T1 - Pathogenesis of idiopathic pulmonary fibrosis and its clinical implications
AU - Spagnolo, Paolo
AU - Rossi, Giulio
AU - Cavazza, Alberto
PY - 2014
Y1 - 2014
N2 - Idiopathic pulmonary fibrosis (IPF) is the most common and lethal form of idiopathic interstitial pneumonia. The disease is thought to arise following an aberrant reparative response to recurrent alveolar epithelial cell injury leading to progressive loss of function. The median survival time is 3-5 years from diagnosis. Cigarette smoking, exposure to organic and inorganic dust and genetic factors have been shown to increase the risk of disease, although the cause of IPF remains elusive and its pathogenesis incompletely understood. In the last decade, several clinical trials evaluating novel therapies for IPF have been conducted but the results have been mostly disappointing. Conversely, compounds that target anti-fibrotic and growth factor pathways have been proven effective in slowing functional decline and disease progression. These promising results notwithstanding, truly effective therapeutic strategies will likely require combinations of drugs in order to target the multitude of pathways involved in disease pathogenesis.
AB - Idiopathic pulmonary fibrosis (IPF) is the most common and lethal form of idiopathic interstitial pneumonia. The disease is thought to arise following an aberrant reparative response to recurrent alveolar epithelial cell injury leading to progressive loss of function. The median survival time is 3-5 years from diagnosis. Cigarette smoking, exposure to organic and inorganic dust and genetic factors have been shown to increase the risk of disease, although the cause of IPF remains elusive and its pathogenesis incompletely understood. In the last decade, several clinical trials evaluating novel therapies for IPF have been conducted but the results have been mostly disappointing. Conversely, compounds that target anti-fibrotic and growth factor pathways have been proven effective in slowing functional decline and disease progression. These promising results notwithstanding, truly effective therapeutic strategies will likely require combinations of drugs in order to target the multitude of pathways involved in disease pathogenesis.
KW - Treatment
UR - http://www.scopus.com/inward/record.url?scp=84904276966&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84904276966&partnerID=8YFLogxK
U2 - 10.1586/1744666X.2014.917050
DO - 10.1586/1744666X.2014.917050
M3 - Article
C2 - 24953006
AN - SCOPUS:84904276966
VL - 10
SP - 1005
EP - 1017
JO - Expert Review of Clinical Immunology
JF - Expert Review of Clinical Immunology
SN - 1744-666X
IS - 8
ER -