Uncovering the facts about pulmonary fibrosis

In recognition of Pulmonary Fibrosis Awareness Month, MultiCare Pulmonologist Venkat Rajasurya, MD, answers common questions about the disease to encourage people to recognize the causes and symptoms and when to seek treatment. With early diagnosis and treatment, people with pulmonary fibrosis can greatly improve their quality of life.

Q: What is pulmonary fibrosis?

Pulmonary fibrosis (PF) is a debilitating and progressive chronic lung disease that involves the scarring and/or inflammation of lung tissue. Over time, the progressive scar tissue buildup can block the movement of oxygen from the lungs into the blood stream and make it difficult for oxygen to reach vital tissues and organs.

Q: What are the symptoms of pulmonary fibrosis?

Major symptoms include shortness of breath, tiredness and a dry, hacking cough. The course of pulmonary fibrosis and the severity of symptoms are unpredictable and can vary considerably from person to person. Some people become ill quickly with severe disease. Others have moderate symptoms that worsen more slowly and gradually over time.

Q: What are the causes of pulmonary fibrosis?

There are various causes of pulmonary fibrosis, including occupational exposure to sand dust, asbestos fibers, hard metal dusts, coal dust or grain dust; environmental factors such as bird droppings; prior radiation therapy for lung or breast cancer; and certain medications and medical conditions such as rheumatoid arthritis, sarcoidosis, scleroderma, lupus and dermatomyositis.

However, in most cases, the cause is never found. Pulmonary fibrosis with no known cause is referred to as idiopathic pulmonary fibrosis (IPF). Age over 50, male gender, acid reflux disease and cigarette smoking are also considered to increase the risk of developing of IPF.

Q: What is the prevalence of pulmonary fibrosis?

The Pulmonary Fibrosis Foundation explains that IPF affects one in 200 US adults over the age of 65. More than 200,000 Americans are currently living with IPF, and each year, there are over 50,000 new diagnoses and an estimated 40,000 deaths. A recent survey showed that most Americans (86%) are unaware of IPF symptoms, which makes early diagnosis and treatment difficult.

Q: How is IPF diagnosed?

The diagnosis of IPF requires exclusion of other known causes of interstitial lung disease with routine laboratory tests and either definite radiographic features on CT chest or with a lung biopsy, which is performed by a thoracic surgeon. As part of the work up, a lung function test is routinely done to measure the lung capacity and a walk test is done to evaluate the need for supplemental oxygen use at home.

Q: How is IPF treated?

Unfortunately, there is no cure for IPF, but antifibrotic drugs such as nintedanib (Ofev) and pirfenidone (Esbriet), both of which have been approved by FDA, have shown in clinical trials to slow the progression of scarring in the lungs. Patients with IPF are frequently referred to a pulmonary rehabilitation program, can help improve symptoms and quality of life. As the scarring of the lung tissue in IPF cannot be reversed, lung transplantation has shown to improve survival in these patients as well.

Q: How does pulmonary rehabilitation help in IPF?

Pulmonary rehabilitation is a comprehensive intervention that includes supervised exercise, education, lifestyle counseling and group support. The goal of pulmonary rehabilitation is to maximize levels of function and quality of life of individuals suffering chronic lung diseases such as IPF. IPF can cause the muscles to weaken, and the exercise program helps in re-strengthening the muscles involved in breathing and in moving, thereby helping to reduce fatigue and improve the level of functioning.

Q: What is the association between pulmonary fibrosis and COVID-19?

Patients with IPF are at higher risk for developing serious complications if infected with COVID-19. COVID-19 vaccination is very safe and is strongly recommended in all patients with IPF. It is also important to know that patients who develop severe lung injury from COVID-19 pneumonia may develop pulmonary fibrosis as a late complication.

Learn more about pulmonary fibrosis and other respiratory conditions treated at MultiCare.