Pulmonary Fibrosis

Pulmonary fibrosis is a lung disease that occurs when lung tissue is damaged or scarred. It causes a scarring and thickening that creates stiffness within the lung tissue. Over time, this condition makes it difficult to breathe and reduces the amount of oxygen entering the bloodstream.

There is no cure as lung damage caused by pulmonary fibrosis cannot be repaired. Treatments may help to manage symptoms and slow the progression of the disease. Early detection and intervention can help improve quality of life.

Pulmonary fibrosis is an incurable lung disease caused by scarring of the lung tissue and alveoli (tiny air sacs in your lungs). The scarring creates thickened, stiff tissue making it increasingly hard for your lungs to function properly. The decrease in lung function limits your ability to move oxygen in and remove carbon dioxide out of your bloodstream. It also makes it more difficult to expand your lungs, which makes it harder for you to breathe, causing shortness of breath.

Over time, the lung damage increases, causing routine tasks to be difficult. Lung damage caused by pulmonary fibrosis is not repairable. Treatment, including medication and therapies, can help decrease the rate of fibrosis, lessen symptoms and improve quality of life.

Symptoms may develop quickly or over a period of years. Typically, pulmonary fibrosis symptoms develop gradually and worsen over time. Common symptoms include:

• Chronic, dry cough
• Fatigue
• Shortness of breath, especially after activity
• Unexplained weight loss

As the disease progresses, you may experience symptoms including:

• Clubbing (widening and rounding of the tips of your fingers and toes)
• Cyanosis (skin discoloration due to a lower level of oxygen in your bloodstream)

Why some people have pulmonary fibrosis is not always known. The disease can develop for several known reasons, including:

• Autoimmune diseases such as lupus, scleroderma, rheumatoid arthritis and Sjogren’s syndrome
• Certain medications
• Exposure to environmental factors, such as asbestos, silica dust, coal dust or mold
• Family history
• Infections
• Radiation therapy

In many people the exact reason for the disease is often unknown. When the cause is unknown, the condition is referred to as idiopathic pulmonary fibrosis. But certain risk factors may make you more likely to develop pulmonary fibrosis, including:

• Autoimmune diseases
• Biologically male
• Emphysema
• Genetics
• Older age (often between ages 50 and 70)
• Radiation therapy
• Smoking
• Work environment with exposure to toxic dust or fumes

Diagnosing pulmonary fibrosis can be challenging due to the gradual onset of symptoms that often look like other respiratory conditions. If you have symptoms, speak with your healthcare team. In addition to asking you about your medical and family history and a physical exam, a member of your care team may order one or more tests such as:

• Biopsy
• Blood tests
• Imaging tests including a chest X-ray, CT scan or echocardiogram
• Pulmonary function tests

At this time, there is no cure for pulmonary fibrosis and lung damage is not reversible. Early detection is key as medical interventions can help improve long-term outcomes. There are treatments to ease symptoms, slow disease progression and improve quality of life including:

• Medication
• Oxygen therapy (supplemental oxygen)
• Pulmonary rehabilitation

For some patients with pulmonary fibrosis, a lung transplant may be an option. Additionally, there are clinical trials where new medications are being tested and current drugs are looked at for new uses. Ask a member of your healthcare team if you’re eligible for a clinical trial.

The University of Kansas Health System’s pulmonary care team provides advanced care for a complete range of respiratory conditions including pulmonary fibrosis. Our specialists, advanced practice providers and nurses deliver the high-quality care you and your loved ones expect and trust.

Our team includes more than 40 board-certified physicians dedicated to multiple subspecialty areas, including pulmonary fibrosis. Our team’s extensive training and knowledge allow us to tailor your care to your unique needs.