What is Drug-Induced Pulmonary Fibrosis?
Pulmonary fibrosis (PF) is part of a larger group of more than 200 interstitial lung diseases (also known as ILDs) that are characterized by inflammation and/or scarring in the lung. In ILDs, the injury and damage occurs in the walls of the air sacs of the lung, as well as in the tissue and space around these air sacs. When an interstitial lung disease includes scar tissue in the lung, we call it pulmonary fibrosis.
Some medications can cause pulmonary fibrosis. Drugs used to treat cancer (chemotherapy drugs like bleomycin), drugs used to treat abnormal heart rhythms (such as amiodarone), drugs used to treat inflammatory conditions (such as methotrexate), and an antibiotic used to treat urinary tract infections (nitrofurantoin) are some of the better known drugs that can cause injury, inflammation, and scarring in the lungs. Numerous other drugs have been found to cause PF in some cases. It is not known why some people who use these drugs develop pulmonary fibrosis, while most do not.
What are the Symptoms of Drug-Induced Pulmonary Fibrosis?
Early in the disease, people with drug-induced pulmonary fibrosis may not experience symptoms or might have a bothersome cough. As the disease progresses, breathlessness during exercise and daily activities can be common. Fatigue, depression, or anxiety are also commonly experienced by people living with pulmonary fibrosis.
How is Drug-Induced Pulmonary Fibrosis Diagnosed?
When a doctor or other healthcare provider suspects that a patient has ILD, they will collect information about the patient’s medical and personal history, work and home environment, hobbies, and illness that may be present in the family (see: Diagnosis). This can help a doctor identify exposures or other diseases that might have caused lung injury and scarring. The doctor will also often order pulmonary function tests, a chest x-ray, blood work, and a high-resolution CT scan.
Pulmonary function tests measure how much air the lungs can hold, and how the lungs are working overall. Scarring can cause the lungs to shrink, and it can also make them stiff and unable to fully expand. This means the lungs are able to hold less air. Scar tissue may also affect the ability of the lungs to transfer oxygen to the bloodstream.
A high-resolution computed tomography (HRCT) scan is a special type of x-ray that shows fine detail of the lung tissue. On a CT scan (also known as CAT scan) healthy lung tissue looks nearly black, and scar tissue and inflammation appear grey or white.
Drug-induced pulmonary fibrosis can be challenging to diagnose, as the relationship between a particular drug and other underlying conditions that can affect the lungs (such as rheumatoid arthritis) can be complicated. If a medication is suspected to have caused pulmonary fibrosis, the medication history, including when it was started and stopped, dosing, and other medications taken at the same time, is a very important part of the evaluation.
How is Drug-Induced Pulmonary Fibrosis Treated?
Cessation of the drug that caused the pulmonary fibrosis is often indicated, though this does not always have an effect on the patient’s immediate condition. Corticosteroid medication can be effective on a temporary basis.
pulmonary rehabilitation, and management of symptoms are important treatment options for many types of pulmonary fibrosis, depending on how severe the disease is. Lung transplantation has been an option for a few patients with drug-induced pulmonary fibrosis.
Drug-induced pulmonary fibrosis can be progressive, though this varies from person to person and depends on the drug that caused the pulmonary fibrosis. Some patients may experience years of stability, while others may experience a rapid progression of disease. For patients who have progressive fibrosis (worsening scarring) as part of their disease, their pulmonologist might consider the use of an anti-fibrotic medication to slow the scarring.
What Should I Do Next?
If you have been recently diagnosed with pulmonary fibrosis, consider making an appointment with a pulmonologist who has experience caring for patients with PF. A knowledgeable team of experts will help make sure you receive an accurate diagnosis and
the most up-to-date treatments and management recommendations. To assist you in finding pulmonologists nearest to your home who provide expert care to patients with PF, the Pulmonary Fibrosis Foundation established the PFF Care Center Network, which includes 68 medical centers throughout the United States. You can search for the PFF Care Center Network medical center closest to
We also recommend that you join a pulmonary fibrosis support group. Connecting with other individuals facing the same illness can help you and your family feel less alone in your journey with pulmonary fibrosis. Support groups can supplement the care
you receive from your healthcare team by providing emotional support and education. You can find a list of support groups
Support groups can help those living with pulmonary fibrosis to:
- Learn about their disease and available treatments
- Feel supported by others who share similar experiences living with PF
- Learn to navigate the healthcare system more effectively
- Improve coping skills, connect with helpful resources, and more
Find reliable information and trusted resources that can help you learn about pulmonary fibrosis and live better with PF.
Pulmonary Fibrosis Information GuideOur comprehensive guide provides reliable information about pulmonary fibrosis, the diagnostic process, treatment options, and more.
Oxygen Basics BookletThis booklet provides an in-depth overview of the the basics of supplemental oxygen, including equipment, getting started, Medicare information, and traveling.
Schwarz MI, King TE. Interstitial Lung Disease. Fifth Edition.Shelton, CT: People’s Medical Publishing House-USA; 2011. 637-658 p.