What is IPF and what are the potential risk factors?

Idiopathic pulmonary fibrosis (IPF) is a specific and the most common form within a heterogeneous group of idiopathic interstitial pneumonias of unknown aetiology, affecting the lung parenchyma. IPF is a chronic, progressive and debilitating disease, characterised by inflammation and scarring of lung tissue resulting in loss of lung function and impaired gas exchange over time.1,2

 

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Generally, IPF is more common in men and it tends to develop later in life.3 A number of other patient-related and environmental risk factors have been proposed (see table below), although the precise aetiology of the disease is unknown.

overview of riskfactors

There are limited treatment options available for IPF and the clinical course is variable. Most patients face a gradual deterioration, although some may progress rapidly or be subject to episodes of rapid deterioration, known as acute exacerbations.6,7

Overall prognosis in IPF patients is poor, with a median survival of 2-3 years after diagnosis.1 This survival rate is even lower than that of several common cancers (see graphic below).8

survival is poor

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References: 
  1. Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788–824.
  2. American Thoracic Society, et al. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 2002;165:277–304.
  3. Meltzer EB, et al. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008;3:8.
  4. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000;161:646–664.
  5. Baumgartner KB, et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Collaborating Centers. Am J Epidemiol 2000;152:307–315.
  6. Ley B, et al. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;183:431–440.
  7. Kim DS, et al. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006;3:285–292.
  8. Vancheri C, et al. Idiopathic pulmonary fibrosis: a disease with similarities and links to cancer biology. Eur Respir J 2010;35:496–504.