TY - JOUR T1 - The Fibroproliferative Response in ARDS: Mechanisms and Clinical Significance JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/09031936.00196412 SP - erj01964-2012 AU - Ellen L. Burnham AU - William J. Janssen AU - David W.H. Riches AU - Marc Moss AU - Gregory P. Downey Y1 - 2013/01/01 UR - http://erj.ersjournals.com/content/early/2013/03/20/09031936.00196412.abstract N2 - The acute respiratory distress syndrome (ARDS) continues to be a major health care problem, affecting more than 190,000 people in the US annually with a mortality of 27–45% depending on the severity of the illness and co-morbidities. 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 characterized 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 will review 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. ER -