National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Idiopathic pulmonary fibrosis

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Other Names:
Fibrosing alveolitis, cryptogenic; Familial idiopathic pulmonary fibrosis; Fibrocystic pulmonary dysplasia; Fibrosing alveolitis, cryptogenic; Familial idiopathic pulmonary fibrosis; Fibrocystic pulmonary dysplasia; Fibrosing alveolitis See More
Categories:
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

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
30%-79% of people have these symptoms
Bronchiectasis
Permanent enlargement of the airways of the lungs
0002110
Clubbing of fingers
Clubbed fingers
Finger clubbing
Clubbing (hands)
[ more ] 		0100759
Cough
Coughing
0012735
Crackles 0030830
Exertional dyspnea 0002875
Gastroesophageal reflux
Acid reflux
Acid reflux disease
Heartburn
[ more ] 		0002020
Ground-glass opacification on pulmonary HRCT 0025179
Honeycomb lung 0025175
Pulmonary fibrosis 0002206
Reticular pattern on pulmonary HRCT 0025390
5%-29% of people have these symptoms
Pulmonary insufficiency 0010444
Percent of people who have these symptoms is not available through HPO
Alveolar cell carcinoma 0006519
Autosomal dominant inheritance 0000006
Cirrhosis
Scar tissue replaces healthy tissue in the liver
0001394
Clubbing
Clubbing of fingers and toes
0001217
Increased circulating antibody level 0010702
Pneumonia 0002090
Pulmonary arterial hypertension
Increased blood pressure in blood vessels of lungs
0002092
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Last updated: 7/1/2020
In the past, the goals of treating idiopathic pulmonary fibrosis (IPF) have been to prevent more lung scarring, relieve symptoms, maintain the ability to be active, and improve the quality of life.[2] More recently, pirfenidone (an anti-fibrotic drug) has been approved to treat people with mild-to-moderate IPF in the European Union, Canada, and Asia.[3] The U.S. Food and Drug Administration (FDA) has granted Breakthrough Therapy Designation for pirfenidone and nintedanib, due to trials suggesting they slow the progression of IPF.[4] Several other drugs are being studied as potential treatments including cotrimoxazole, thalidomide, sildenafil, andimatinib mesylate. However, more research is needed to determine their safety and effectiveness.[3]

Most affected people need oxygen therapy at some point to increase oxygen levels in the bloodstream. Oxygen therapy can reduce breathlessness and allow people to be more active. Some people benefit from pulmonary rehabilitation, used for people with chronic lung diseases.

People with IPF may eventually need a lung transplant. This is more likely in younger patients (under 65) with severe disease who have not responded to other treatments, and who don't have other serious medical problems. Some consider lung transplants for people over 65 who don't have other serious medical problems.

For many years, corticosteroids (such as prednisolone) along with immunosuppressive drugs (such as azathioprine) were used to treat IPF. Sometimes an additional drug called N-acetylcysteine has also been used. These drugs were recommended based on the theory that generalized inflammation was a major part of IPF. However, the drugs were often ineffective and there has not been evidence that they improve long-term survival.

Gastroesophageal reflux may be treated with standard medications. Some studies have shown longer survival times and lower fibrosis scores in people receiving treatment for gastroesophageal reflux.[3]
Last updated: 7/30/2014

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

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:[5]
  • 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.[6]

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:[6]
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

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnosis includes other idiopathic interstitial pneumonias, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis (see these terms) and other environmental (sometimes occupational) exposures.
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Idiopathic pulmonary fibrosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Social Networking Websites

  • RareConnect has an online community for patients and families with this condition so they can connect with others and share their experiences living with a rare disease. The project is a joint collaboration between EURORDIS (European Rare Disease Organisation) and NORD (National Organization for Rare Disorders).

Organizations Providing General Support

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • 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

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions.
  • 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.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • I have been diagnosed with idiopathic pulmonary fibrosis. There is no cure. The only prognosis I have found on the internet is 3 - 5 years of life after first diagnosis. Yet I read on blogs that many live longer without a lung transplant. Do you have more info? See answer

  • My wife was recently diagnosed with idiopathic pulmonary fibrosis. She is being treated with N-Acetylcysteine, an anti-oxidant supplement.  Is there a newer therapy or treatment for this condition? See answer


  1. What Is Idiopathic Pulmonary Fibrosis?. NHLBI. September 20, 2011; http://www.nhlbi.nih.gov/health/health-topics/topics/ipf/. Accessed 7/30/2014.
  2. Idiopathic Pulmonary Fibrosis. National Heart, Lung, and Blood Institute (NHLBI). 2007; http://www.nhlbi.nih.gov/health/dci/Diseases/ipf/ipf_whatis.html. Accessed 12/9/2008.
  3. Idiopathic pulmonary fibrosis. NORD. May 7, 2014; https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/432/viewAbstract. Accessed 7/30/2014.
  4. NINTEDANIB AND PIRFENIDONE GRANTED BREAKTHROUGH THERAPY DESIGNATION. Pulmonary Fibrosis Foundation. July 17, 2014; http://www.pulmonaryfibrosis.org/our-role/news-media/viewannouncement/nintedanib-and-pirfenidone-granted-breakthrough-therapy-designation. Accessed 7/30/2014.
  5. Talmadge E King, Jr. Treatment of idiopathic pulmonary fibrosis. UpToDate. Waltham, MA: UpToDate; July, 2014; Accessed 7/31/2014.
  6. 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.