title: Emerging drugs for idiopathic pulmonary fibrosis.
abstract: BACKGROUND
  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and usually lethal lung disorder of unknown etiology. The disease is characterized by alveolar epithelial cell injury, formation of activated fibroblasts/myofibroblasts foci and finally by the exaggerated accumulation of extracellular matrix with the subsequent destruction of the lung architecture. The long-term survival is distinctly poor, with only a 20-30% survival 5 years after the time of diagnosis. Actually, regardless of extensive research, no current therapy has been shown to either reverse or stop the progression of this disease.
  
  
  METHODS
  The authors searched the Medline database from January 1990 to December 2010 using search terms 'pulmonary fibrosis', 'fibrosing alveolitis' and 'usual interstitial pneumonia'. Several subsets were included: definition and epidemiology, risk factors, clinical behavior, pathogenesis and therapy. For the section of IPF treatment, the authors examined all relevant studies including randomized controlled trials, cohort studies, case-control studies and cross-sectional studies. In this review, the authors describe the current therapeutic approaches, the ongoing clinical trials and some future options based on stem cells, lung bioengineering and microRNAs.
  
  
  CONCLUSIONS
  The treatment of IPF represents one of the greatest challenges confronting respiratory medicine and, currently, there is no effective therapeutic option for IPF. Perhaps some of the drugs that are under evaluation in clinical trials will slow the decline of the pulmonary function tests or hopefully stabilize some patients. Nonetheless, it appears clear that new therapeutic approaches are urgently needed.
authors.affiliation: Instituto Nacional de Enfermedades Respiratorias, Tlalpan 4502, CP 14080, México DF, México. mselmanl@yahoo.com.mx
authors.forename: Moisés
authors.initials: M
authors.lastname: Selman
authors.forename: Annie
authors.initials: A
authors.lastname: Pardo
authors.forename: Luca
authors.initials: L
authors.lastname: Richeldi
authors.forename: Stefania
authors.initials: S
authors.lastname: Cerri
mesh_terms: D000818:Animals; D016022:Case-Control Studies; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy*; D016032:Randomized Controlled Trials as Topic