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

Treatment

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How might idiopathic pulmonary fibrosis be treated?

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.[1] 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.[2] 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.[3] 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.[2]

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.[2]
Last updated: 7/30/2014

References
  1. 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.
  2. Idiopathic pulmonary fibrosis. NORD. May 7, 2014; https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/432/viewAbstract. Accessed 7/30/2014.
  3. 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.


Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Idiopathic pulmonary fibrosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
  • 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".

Medical Products

The medication(s) listed in the table(s) below have been approved by the Food and Drug Administration (FDA) for treatment of this condition. The FDA Office of Orphan Products Development designates "orphan products" for those that treat rare diseases affecting fewer than 200,000 Americans. The table(s) below may not be an exhaustive list of drugs or products used to treat this condition. There may be other products available that are not considered orphan products. To search for all FDA approved drugs, visit Drugs@FDA. You can find orphan products used to treat other conditions by searching the Orphan Drug Product Designation database.


Generic Name pirfenidone
Trade Name
(Manufacturer Name)
Esbriet
(InterMune, Inc.)
Indication
The FDA has approved this product to be used in this manner.
Treatment of idiopathic pulmonary fibrosis.
More Information about this product Drug Information Portal

Generic Name nintedanib
Trade Name
(Manufacturer Name)
Ofev
(Boehringer Ingelheim Pharmaceuticals, Inc.)
Indication
The FDA has approved this product to be used in this manner.
Treatment of idiopathic pulmonary fibrosis.
More Information about this product Drug Information Portal

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.