Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

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.

Your Question

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?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is idiopathic pulmonary fibrosis?

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

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.[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

Are there any research studies investigating new therapies for the treatment of idiopathic pulmonary fibrosis?

Yes. The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. Currently, there are a number of clinical trials enrolling individuals with idiopathic pulmonary fibrosis. Many of these trials are investigating new therapies. To find these trials, click here. After you click on a study, review its "eligibility" criteria to determine its appropriateness. Use the study‚Äôs contact information to learn more. Check this site often for regular updates.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if your wife is eligible for any clinical trials.  

Patient Recruitment and Public Liaison Office
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 800-411-1222
Fax: 301-480-9793
Email: prpl@mail.cc.nih.gov
Web site: http://clinicalcenter.nih.gov/  

If your wife is interested in enrolling in a clinical trial, she can find helpful general information on clinical trials at the following ClinicalTrials.gov Web page.
http://clinicaltrials.gov/ct2/info/understand  

A tutorial about clinical trials that can also help answer her questions can be found at the following link from the National Library of Medicine:
http://www.nlm.nih.gov/medlineplus/tutorials/cancerclinicaltrials/htm/lesson.htm  

Resources on many charitable or special-fare flights to research and treatment sites and low-cost hospitality accommodations for outpatients and family members, as well as ambulance services, are listed on the Web site of the Office of Rare Diseases (ORD), part of the National Institutes of Health.
http://rarediseases.info.nih.gov/Resources.aspx?PageID=8  
Last updated: 12/9/2008

References
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.