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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
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

Idiopathic pulmonary fibrosis (IPF) is a condition in which tissues in the lungs become thick and stiff, or scarred, over time. The lungs then lose their ability to move oxygen to the brain and other parts of the body. Common symptoms include shortness of breath and a dry, hacking cough. In some cases fibrosis happens quickly, while in others, the process is much slower. Sometimes the disease stays the same for years. The condition is 'idiopathic' because the cause is unknown. When multiple family members are affected, it is called familial IPF. Many people with this condition live for about 3-5 years after the diagnosis. The most common cause of death is respiratory failure.[1]
Last updated: 7/30/2014

References

  1. What Is Idiopathic Pulmonary Fibrosis?. NHLBI. September 20, 2011; http://www.nhlbi.nih.gov/health/health-topics/topics/ipf/. Accessed 7/30/2014.
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Basic Information

  • The Mayo Clinic provides information about Idiopathic pulmonary fibrosis. Click on the above link to access this information.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Heart, Lung, and Blood Institute (NHLBI) provides leadership for a national program in diseases of the heart, blood vessels, lung, and blood; blood resources; and sleep disorders. Since October 1997, the NHLBI has also had administrative responsibility for the NIH Woman's Health Initiative. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Idiopathic pulmonary fibrosis. Click on the link to view a sample search on this topic.

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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.