Pulmonary fibrosis is a group of serious lung diseases that affect the respiratory system. Pulmonary fibrosis scars and thickens lung tissue. It impacts the connecting tissue in the lung and the alveoli (air sacs inside the lungs).
Many things (like smoking) can lead to pulmonary fibrosis. Often, the cause remains unknown (idiopathic pulmonary fibrosis). Some types of pulmonary fibrosis may run in families.
Certain risk factors, like smoking, may make it more likely you could develop pulmonary fibrosis. But even having one or more risk factors doesn’t mean you’ll definitely get the disease one day.
Other risk factors of pulmonary fibrosis include:
Older age: Most people who get pulmonary fibrosis develop it in the second half of life, between ages 50 and 70.
Male biological sex: Pulmonary fibrosis affects more men than women. However, cases in women have risen in recent years.
Smoking: Cigarette smoking increases your risk of getting pulmonary fibrosis.
Working around dust or fumes: Regularly breathing in chemicals or hazardous substances can damage the lungs. Farmers, ranchers, hairdressers, stone cutters/polishers and metal workers may be at increased risk.
Other medical conditions: In some cases, another medical condition (such as the autoimmune disease rheumatoid arthritis or a viral infection) leads to pulmonary fibrosis.
Other factors: Radiation exposure, such as radiation therapy to treat cancer, can damage lung tissue. So can some medications, including chemotherapy and certain heart medications.
Pulmonary fibrosis symptoms include:
Breathing in short, shallow spurts.
Dry cough that doesn’t go away.
Fatigue (extreme tiredness, no matter how much you sleep).
Shortness of breath, especially during or soon after you exercise.
Weight loss that’s not on purpose or easily explained.
As the disease progresses, some people experience:
Clubbing, fingertips or toes that look different, such as wider or more round.
Cyanosis, bluish skin (in fair-skinned people) or gray or white skin around the mouth or eyes (in dark-skinned people) from too little oxygen in the blood.
Blood tests: Your provider may order blood tests to rule out other illnesses or reasons for your symptoms. Lab tests can also help providers track disease progression (how it affects your body over time) after diagnosis.
Imaging tests: A chest X-ray or a CT scan may help rule out other lung-related illnesses. These pictures can clearly show lung scarring and may confirm a pulmonary fibrosis diagnosis.
Breathing tests: These tests are also called pulmonary function tests. Different devices measure lung function and capacity (how well your lungs work).
Oxygen desaturation study: This test measures oxygen levels in your blood. You walk for six minutes with a probe attached to your finger or forehead.
Biopsy: A surgeon removes a small lung tissue sample through a small incision in the ribs. Providers sometimes perform a lung biopsy to confirm a pulmonary fibrosis diagnosis.
Your provider may recommend one or more treatments:
Medication: Two medications like pirfenidone (Esbriet) and nintedanib (OFEV) may slow down lung scarring. These medications can help preserve lung function.
Oxygen therapy: Giving your body extra oxygen helps you breathe more easily. It may also increase your energy and strength.
Pulmonary rehabilitation: Staying active in this special exercise program may improve how much (or how easily) you can do everyday tasks or activities.
Lung transplant: A lung transplant replaces one or both diseased lungs with a healthy lung (or lungs) from a donor. It offers the potential to improve your health and quality of life. A lung transplant is major surgery, and not everyone is a candidate. Ask your provider if you may be eligible for a lung transplant.