Idiopathic pulmonary fibrosis (IPF) is a chronic disease causing inflammation and fibrosis (scarring) of tissue in the lungs.
At UVA, our experience in the rare and complex disorders that constitute pulmonary fibrosis means that we can develop an approach to your unique condition with accuracy and precision.
How Research Helps Treatment
Our investigational research and clinical trials affords us the ability to discover new approaches to pulmonary fibrosis and the chance to offer you the latest treatments available.
Get a glimpse of the medical horizon: Find out more about our current research in pulmonary fibrosis.
Your Treatment Options for Pulmonary Fibrosis
Treatment depends on the cause of interstitial lung disease (ILD); a correct diagnosis is critical. Treatment aims to reverse inflammation and stop scar formation. In some types of ILD, damage to the lungs is permanent and can’t be reversed, but treatment will help to ease symptoms and improve quality of life.
Treatment will be based on your specific condition and symptoms.
Medications can help to slow the progression of ILD. Options include:
- Pirfenidone and nindetanib, newly approved drugs for the treatment of idiopathic pulmonary fibrosis (UVA was one of the first centers that studied the effectiveness of pirfenidone in IPF, leading to the approval of the drug.)
- Supplemental oxygen therapy; in people with low oxygen saturation, this treatment increases the amount of oxygen in the blood, improves symptoms and allows patients to live longer and healthier lives
- In some cases, medications that suppress the immune system (interfere with the process of inflammation and fibrosis)
Pulmonary rehabilitation helps you manage shortness of breath. Rehabilitation is tailored to your needs but may include:
- Breathing techniques and coping strategies
- Support from others
Learning to live with an illness can be difficult. You may benefit from psychological counseling that can help you better manage your life. Counseling can be individual or in a support group.
If you smoke, your doctor will advise you about the most effective programs to help you quit.
If other treatments fail, a lung transplant may be necessary. A transplant can improve both the quality and length of your life.
Preventing Lung Disease
Not all ILDs can be prevented. To help reduce your exposure to substances associated with some ILDs:
- Talk to your doctor about ways to help you quit smoking
- Avoid lung irritants whenever possible
- Follow occupational guidelines to protect your lungs at work
- Use proper protection when exposed to harmful chemicals, dust or animal droppings
What Causes IPF?
'Idiopathic' means the cause is not known.
Researchers think that IPF is an exaggerated and uncontrolled inflammatory response. This produces the scar tissue. What starts the cycle is not known. Over time, scarring surrounds the thin walled air sacs in the lungs. This makes the tissue thicker and stiffer. As a result, breathing becomes difficult. The lungs gradually lose their ability to pass oxygen to the rest of the body.
Risk Factors for IPF
IPF occurs most often in males and people over 50. Other factors below may increase your chance of IPF include:
- Sex: male
- Age: 50 and older
- Cigarette smoking
- Viral infection
- Occupational exposures to dusts containing wood, metal, silica, bacteria, animal proteins, aerosol sprays, gases and fumes
- Medications such as nitrofurantoin, sulfasalazine, amiodarone, propranolol, methotrexate, cyclophosphamide, bleomycin
- Gastroesophageal reflux disease (GERD)
- Other family members with IPF
Over time, the symptoms get worse. This makes daily activities difficult. People with IPF gradually start to have some or all of these symptoms:
- Shortness of breath, at first only during or after physical activity, but later also when resting
- Dry cough
- Gradual weight loss
- Clubbing (enlargement of the fingertips or sometimes the toes)
Content was created using EBSCO’s Health Library. Edits to original content made by Rector and Visitors of the University of Virginia. This information is not a substitute for professional medical advice.