There are many forms of pulmonary fibrosis and treatment options vary depending on the underlying cause of the fibrosis. Not all types of interstitial lung disease or pulmonary fibrosis will require treatment as they may remain stable without treatment for many years. Some types of interstitial lung disease and pulmonary fibrosis respond to steroids such as prednisone while others do not. It is important to discuss your specific diagnosis with your physician because the specific type of ILD/pulmonary fibrosis will determine the type of treatment options available.
There are two anti-fibrotic medications approved by the FDA. Currently they are only approved for the treatment of IPF (discussed below). The drug companies that manufacture the two medications are in the process of studying whether these drugs would be beneficial to patients with other types of pulmonary fibrosis including non-idiopathic pulmonary fibrosis.
Therapies for Idiopathic Pulmonary Fibrosis (IPF)
There are two FDA approved therapies for IPF—Esbriet and Ofev. Each of these medications has been independently shown in at least two large randomized placebo-controlled trials to significantly slow the rate of lung function decline in patients with IPF. Neither of these medications cures or reverses the fibrosis. Therefore, early diagnosis and treatment is critical in the management of IPF.
Novel drug targets for idiopathic pulmonary fibrosis. Sgalla G, Cocconcelli E, Tonelli R, Richeldi L. Expert Rev Respir Med. 2016 Feb 26:1-13. [Epub ahead of print] PMID:26854382
Lung transplantation is the only known cure for IPF. However, not everyone with IPF will be a suitable candidate for lung transplantation. Lung transplantation referral is initiated after a discussion with your treating pulmonologist. However, the decision about a patient’s candidacy for transplant is made by the transplant center to which a patient is referred. There are numerous lung transplant centers around the United States. The lung transplant centers closest to the University of Rochester are The Cleveland Clinic, The University of Pittsburgh Medical Center, and Columbia University in New York City. Insurance providers often have preferred centers for transplant referral.
Pulmonary rehabilitation (PR) is a broad, outpatient-based program that helps improve the well-being of people who have chronic (ongoing) breathing problems. The program includes exercise training, nutritional counseling, education on lung disease and its management, energy-conserving techniques, breathing strategies, psychological counseling and/or group support. The major goal of PR is to improve the ability to function and quality of life. PR may help improve breathing. Even if you have advanced lung disease, you can still benefit from PR.
If you have an interstitial lung disease like IPF, you may require oxygen therapy to maintain normal oxygen levels. Supplemental oxygen may also help you to stay active and gives you the opportunity to continue to enjoy activities outside of your home. There are several options by which oxygen is delivered. To learn more about oxygen therapy, please click on the following links: