RT Journal Article
SR Electronic
T1 The fibroproliferative response in acute respiratory distress syndrome: mechanisms and clinical significance
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 276
OP 285
DO 10.1183/09031936.00196412
VO 43
IS 1
A1 Ellen L. Burnham
A1 William J. Janssen
A1 David W.H. Riches
A1 Marc Moss
A1 Gregory P. Downey
YR 2014
UL http://erj.ersjournals.com/content/43/1/276.abstract
AB Acute respiratory distress syndrome (ARDS) continues to be a major healthcare problem, affecting >190 000 people in the USA annually, with a mortality of 27–45%, depending on the severity of the illness and comorbidities. Despite advances in clinical care, particularly lung protective strategies of mechanical ventilation, most survivors experience impaired health-related quality of life for years after the acute illness. While most patients survive the acute illness, a subset of ARDS survivors develops a fibroproliferative response characterised by fibroblast accumulation and deposition of collagen and other extracellular matrix components in the lung. Historically, the development of severe fibroproliferative lung disease has been associated with a poor prognosis with high mortality and/or prolonged ventilator dependence. More recent studies also support a relationship between the magnitude of the fibroproliferative response and long-term health-related quality of life. The factors that determine which patients develop fibroproliferative ARDS and the cellular mechanisms responsible for this pathological response are not well understood. This article reviews our current understanding of the contribution of pulmonary dysfunction to mortality and to quality of life in survivors of ARDS, the mechanisms driving pathological fibroproliferation and potential therapeutic approaches to prevent or attenuate fibroproliferative lung disease. Despite protective ventilation, a subset of ARDS survivors develop lung fibrosis contributing to poor quality of life http://ow.ly/q8xCo