Idiopathic pulmonary fibrosis (IPF) is a chronic disease of the lungs. It causes inflammation and scarring of lung tissue.
Idiopathic means the cause is not known.
Something starts uncontrolled inflammation. The inflammation damages tissue and leads to scarring. Over time, the scars surround the thin air sacs in the lungs. They make the sacs thicker and stiffer. Gases like oxygen will have a harder time passing through these sacs. You will have to breathe harder to get enough oxygen. The lungs will worsen until they lose their ability to pass oxygen.
IPF occurs most often in males and people aged 50 years and older. Other factors that may increase your chance of IPF include:
- Cigarette smoking
- Viral infection
- Frequent exposure to dusts containing wood, metal, silica, bacteria, and animal proteins or to aerosol sprays, gases, and fumes
- Gastroesophageal reflux disease (GERD)
- Other family members with IPF
Symptoms get worse over time. They will make daily tasks difficult. People with IPF gradually start to have some or all of these symptoms:
- Shortness of breath during or after physical activity then later also when resting
- Dry cough
- Gradual weight loss
- Enlargement of the fingertips, called clubbing, or sometimes the toes
You will be asked about your symptoms and past health. A physical exam will be done.
Your bodily fluids and tissues may be tested. This can be done with:
- Blood tests
- Lung biopsy—a sample of lung tissue is removed and examined in a lab
Images may be taken of your lungs. This can be done with:
Your lung function may be tested. This can be done with:
- Pulmonary function tests
- An exercise test
There is no known cure. The goal of treatment is to ease symptoms. Options include:
Medicine may help to ease some symptoms. A combination of medicine may be used to:
- Reduce inflammation
- Slow or decrease the immune response
- Slow the progress of scarring
Pirfenidone and nintedanib may help to slow fibrosis. This may slow worsening of lung function
Diet and exercise can play important roles. Education support can also help to adjust to changes. A pulmonary rehabilitation program may include:
- Strength and endurance exercise program
- Educational information and lectures
- Nutrition education and planning
- Emotional and social support
Quit and Avoid smoking
Smoking damages lung tissue. It will make symptoms worse. Tools may help to quit smoking. Inhaling smoke from others can be harmful as well.
There is no proven way to prevent most IPF. Proper protection when working at a high risk job may help.
- Reviewer: Michael Woods, MD
- Review Date: 05/2018 -