Pulmonary Fibrosis

What’s Pulmonary Fibrosis?

Pulmonary fibrosis is a chronic lung disease that causes scarring in the lungs. Over time, this scarring (also called fibrosis) thickens the lung tissue, making it hard for the lungs to work properly. People with pulmonary fibrosis may feel short of breath and have a dry, ongoing cough. It’s more common in older adults and men, but it can affect anyone. In some cases, the cause is unknown—this is called idiopathic pulmonary fibrosis (IPF).

Causes and Risk Factors

While the exact cause of pulmonary fibrosis is sometimes unknown, several factors can increase your risk:

• Exposure to harmful substances (like asbestos or silica)
• Autoimmune diseases (such as rheumatoid arthritis)
• Connective tissue disease or granulomatous disease (like sarcoidosis)
• A family history of lung disease
• Certain medications or radiation treatments
• Aging and history of smoking

How Pulmonary Fibrosis Is Diagnosed

Your doctor may use the following to help diagnose pulmonary fibrosis:

• A review of your medical history and symptoms
• A physical exam
• Lung function tests
• Imaging tests like high-resolution CT scans
• A lung biopsy, in some cases

Signs and Symptoms

Symptoms of pulmonary fibrosis can vary depending on how far the disease has progressed. Early signs may include:

• Shortness of breath, especially during physical activity
• Decreased exercise tolerance
• A dry, persistent cough
• Fatigue or weakness
• Chest discomfort
• Clubbing (widening or rounding of the fingertips or toes)

As the disease advances, symptoms may become more severe and interfere with daily activities. Some people experience a gradual decline, while others notice symptoms worsen more quickly.

In some cases, symptoms can suddenly get worse. This is called an acute exacerbation and requires urgent medical care.

What’s Interstitial Lung Disease (ILD)?

Pulmonary fibrosis is a type of interstitial lung disease (ILD). ILDs affect the thin tissue around the air sacs in your lungs. Scarring in this tissue makes it harder for oxygen to move into your bloodstream. Over time, this can lead to shortness of breath, fatigue, and reduced lung function.

There are more than 200 types of ILD. Other forms include sarcoidosis and hypersensitivity pneumonitis. Diagnosing ILD may involve lung function tests, imaging scans, and sometimes a lung biopsy. Because each type of ILD is different, getting an accurate diagnosis is essential for developing a personalized treatment plan to help manage symptoms and slow the disease.

How Lung Function Is Affected

Scarring makes it harder for your lungs to expand and deliver oxygen. Pulmonary function tests measure how well your lungs work. Over time, the disease can make everyday tasks more difficult. It can also lead to complications like pulmonary hypertension.

Treatment Options

Your care plan depends on the cause and stage of your disease. Common treatments include:

• Medications – Antifibrotic drugs help slow down lung scarring.
• Oxygen therapy – Helps maintain oxygen levels and ease shortness of breath.
• Pulmonary rehabilitation – Combines exercise, breathing techniques, and education.
• Lung transplant – May be an option in advanced cases, and has been shown to increase survival and improve quality of life in patients with end-stage lung disease

At Jackson Health System, our pulmonology team creates personalized care plans to help you manage symptoms and protect your lung health.

Living with Pulmonary Fibrosis

Managing this disease takes teamwork and planning. Here’s how you can stay on track:

• Follow your provider’s care plan
• Bring a family member to appointments for support
• Eat well and stay active if your provider recommends it
• Join a support group for shared experiences and education
• Talk to your provider about counseling or emotional support

Nutrition and Lifestyle Tips

Eating well helps support lung health. Choose balanced meals with fruits, vegetables, whole grains, and lean proteins. Stay hydrated and maintain a healthy weight.

Work, Travel, and Daily Life

This condition may affect your ability to work or travel. Planning ahead and speaking with your employer or provider can help you manage.

Supportive and End-of-Life Care

As the disease advances, focus may shift to comfort and quality of life. Palliative care can help manage symptoms. Advance care planning lets you make your wishes known.

Our Pulmonology Services

Jackson Health System’s pulmonology specialists provide advanced care for complex lung conditions like pulmonary fibrosis. From diagnosis to long-term management, our team is here to support you. Learn more or schedule an appointment jacksonhealth.org/services/pulmonology.