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Diseases

Genetic and Rare Diseases Information Center (GARD)

Idiopathic pulmonary fibrosis


Other Names for this Disease
  • Fibrosing alveolitis, cryptogenic
  • Familial idiopathic pulmonary fibrosis
  • Fibrocystic pulmonary dysplasia
  • Fibrosing alveolitis
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

  • Genetics Home Reference (GHR) contains information on Idiopathic pulmonary fibrosis. This website is maintained by the National Library of Medicine.
  • 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) has information on this topic. NHLBI is part of the National Institutes of Health and supports research, training, and education for the prevention and treatment of heart, lung, and blood diseases.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a 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.
Other Names for this Disease
  • Fibrosing alveolitis, cryptogenic
  • Familial idiopathic pulmonary fibrosis
  • Fibrocystic pulmonary dysplasia
  • Fibrosing alveolitis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.