RT Journal Article
SR Electronic
T1 The pathogenesis of pulmonary fibrosis: a moving target
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1207
OP 1218
DO 10.1183/09031936.00073012
VO 41
IS 5
A1 Wim A. Wuyts
A1 Carlo Agostini
A1 Katerina M. Antoniou
A1 Demosthenes Bouros
A1 Rachel C. Chambers
A1 Vincent Cottin
A1 Jim J. Egan
A1 Bart N. Lambrecht
A1 Rik Lories
A1 Helen Parfrey
A1 Antje Prasse
A1 Carlos Robalo-Cordeiro
A1 Eric Verbeken
A1 Johny A. Verschakelen
A1 Athol U. Wells
A1 Geert M. Verleden
YR 2013
UL http://erj.ersjournals.com/content/41/5/1207.abstract
AB Pulmonary fibrosis is the end stage of many diffuse parenchymal lung diseases. It is characterised by excessive matrix formation leading to destruction of the normal lung architecture and finally death. Despite an exponential increase in our understanding of potentially important mediators and mechanisms, the delineation of primary pathways has proven to be elusive. In this review susceptibility and injurious agents, such as viruses and gastro-oesophageal reflux, and their probable role in initiating disease will be discussed. Further topics that are elaborated are candidate ancillary pathways, including immune mechanisms, oxidative and endoplasmic reticulum stress, activation of the coagulation cascade and the potential role of stem cells. This review will try to provide the reader with an integrated view on the current knowledge and attempts to provide a road map for future research. It is important to explore robust models of overall pathogenesis, reconciling a large number of clinical and scientific observations. We believe that the integration of current data into a “big picture” overview of fibrogenesis is essential for the development of effective antifibrotic strategies. The latter will probably consist of a combination of agents targeting a number of key pathways.