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General Information About IPF

Pulmonary Fibrosis Week

Pulmonary Fibrosis Week

Pulmonary fibrosis is the term used to describe excessive deposition of collagen and other connective tissue matrices in the lung.  This leads to tissue scarring, decreased lung function and oxygenation, increased work of breath, and, ultimately, respiratory failure.  There are many lung disorders characterized by pulmonary fibrosis and these can be elicited or worsened by environmental / occupational exposures (e.g., asbestos), tobacco smoke, infection, or drugs.  Yet in other circumstances, the cause of pulmonary fibrosis is unknown or idiopathic.   This is the case for Idiopathic Pulmonary Fibrosis (IPF), which affects close to 200,000 Americans and many more patients worldwide.  IPF carries a poor prognosis with mortality reaching 50% in 3 years after diagnosis.  Patients with IPF may benefit from oxygen supplementation, pulmonary rehabilitation, the prevention and/or treatment of complications, and even lung transplantation, but there are no FDA-approved drugs known to improve survival.  With the help of basic studies and animal models of pulmonary fibrosis, promising mechanisms of action have been identified for targeting.  These studies have unveiled that some of these patients, not all, have genetic abnormalities that predispose them to the disease.  However, the majority of patients seem to develop a sporadic form of the condition.  Although initial studies pointed to excess inflammation as the main driver of pulmonary fibrosis, more recent studies suggest abnormalities in specific cell types in the lung such the lung fibroblast.  Dysfunction of the epithelium is also suspected to lead to fibroproliferation and scar formation and this has become an important area of investigation.  These and related studies are indeed promising, but have yet to unveil an effective therapy.  Fortunately, IPF has become one of the best studied fibrosing lung disorders.  With multiple basic studies and clinical trials underway, it is anticipated that safe and effective treatments will be available in the not-too-distant future.    Clinical trials are targeting mechanisms of action implicated in the development of disease such as excessive immunity, alterations in cellular signaling, and abnormal cell differentiation.  However, more work needs to be done.   Animal models that better resemble the human condition are very much needed, while multidisciplinary studies evaluating how other organs develop fibrosis should accelerate discovery.  Studies using a comparative biology approach to determine how pulmonary fibrosis develops in other animals and how this knowledge can be used to treat humans are also important.  ATS members and partners are engaged in these and more initiatives, all designed to get us closer to stamping out this devastating disease.


 

Important Facts About Pulmonary Fibrosis

Every day in America:

  • 128,000 people are suffering from PF
  • 130 people will be diagnosed with PF
  • As many people die to PF as to Breast Cancer