Idiopathic pulmonary fibrosis
Other Names for this Disease
- Familial idiopathic pulmonary fibrosis
- Fibrocystic pulmonary dysplasia
- Fibrosing alveolitis
- Fibrosing alveolitis, cryptogenic
- Hamman-Rich disease
Your QuestionMy 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?
We have identified the following information that we hope you find helpful. If you still have questions, please contact us.
Questions on this page
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.
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’s 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.
NIH Clinical Center
Bethesda, Maryland 20892-2655
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.
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:
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.
- What Is Idiopathic Pulmonary Fibrosis?. NHLBI. September 20, 2011; http://www.nhlbi.nih.gov/health/health-topics/topics/ipf/. Accessed 7/30/2014.
- 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.
- Idiopathic pulmonary fibrosis. NORD. May 7, 2014; https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/432/viewAbstract. Accessed 7/30/2014.
- 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.