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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Idiopathic pulmonary fibrosis


Other Names for this Disease

  • Familial idiopathic pulmonary fibrosis
  • Fibrocystic pulmonary dysplasia
  • Fibrosing alveolitis
  • Fibrosing alveolitis, cryptogenic
  • Hamman-Rich disease
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Prognosis

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What is the long-term outlook for people with idiopathic pulmonary fibrosis?

The long-term outlook (prognosis) for people with idiopathic pulmonary fibrosis (IPF) is poor, with only 20-30% of affected people surviving at least 5 years after diagnosis. Several factors have been associated with a shortened survival time, including:[1]
  • older age at diagnosis
  • extensive cigarette smoking
  • lower body mass index (BMI)
  • more severe physiologic impairment
  • greater extent of disease on imaging studies
  • the development of other complications or conditions (eg, pulmonary hypertension, emphysema, and bronchogenic cancer)

The natural history of IPF is not completely understood. While it usually follows a course of progressive deterioration, some people remain stable for extended periods and individual outcomes can vary significantly. Still, long-term survival of IPF is not expected.[2]

It now appears that three potential disease courses exist, but there is not currently a definitive way to predict the course in an affected person. They are:[2]
a) slowly progressive disease (the most common);
b) disease marked by episodes of acute exacerbations; and
c) rapidly progressive disease.

Last updated: 7/31/2014

References
  1. Talmadge E King, Jr. Treatment of idiopathic pulmonary fibrosis. UpToDate. Waltham, MA: UpToDate; July, 2014; Accessed 7/31/2014.
  2. Eric B Meltzer and Paul W Noble. Idiopathic pulmonary fibrosis. Orphanet Journal of Rare Diseases. 2008; 3:8:http://www.ojrd.com/content/3/1/8. Accessed 7/30/2014.


Other Names for this Disease
  • Familial idiopathic pulmonary fibrosis
  • Fibrocystic pulmonary dysplasia
  • Fibrosing alveolitis
  • Fibrosing alveolitis, cryptogenic
  • Hamman-Rich disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.