Pulmonary Fibrosis

Overview

Pulmonary fibrosis is a family of more than 200 different lung diseases characterized by scarring in the lungs. As the disease progresses, scar tissue destroys the healthy lung and makes it hard for oxygen to flow to the blood. When this occurs, you may experience shortness of breath during physical activity.

Pulmonary fibrosis is a part of a larger group of diseases called interstitial lung disease (ILD). ILD is all the conditions where the lungs become inflamed and develop scar tissue. Some ILD conditions do not have scar tissue. If an ILD does have scar tissue in the lung, it is pulmonary fibrosis.

According to the Pulmonary Fibrosis Foundation, pulmonary fibrosis affects 1 out of 200 adults over the age of 60 in the United States. 

Pulmonary fibrosis causes

There are five main categories of causes of pulmonary fibrosis, including:

Drug-induced. Current or previous use of drugs such as amiodarone, nitrofurantoin, chemotherapy, methotrexate may cause pulmonary fibrosis. 

• Radiation-induced. Previous or current radiation treatment to the chest.
• Environmental. Long-term exposure to mold, animals, or other triggers can cause pulmonary fibrosis.
• Autoimmune. Inflamed joints, skin changes on fingers or face, abnormal blood tests, dry eyes, or mouth are causes of pulmonary fibrosis.
• Occupational. Long-term exposure to dusts, fibers, fumes, or vapors such as asbestos, coal, silica, etc. can cause pulmonary fibrosis. 

Environmental and autoimmune causes are the most common causes of pulmonary fibrosis.

When your doctor cannot determine the cause of your pulmonary fibrosis, it is called idiopathic.

Pulmonary fibrosis risk factors

Factors that increase your risk of developing pulmonary fibrosis include: 

• Age. Middle-aged and older adults are most likely to develop pulmonary fibrosis.
• Gender. Men are more likely to develop pulmonary fibrosis than women. 
• Smoking. Smokers are more likely to develop pulmonary fibrosis than nonsmokers. 
• Specific occupations. People who work in mining, farming, construction, or those exposed to other pollutants are more likely to develop pulmonary fibrosis.
• Cancer treatments. People on chemotherapy medication or having radiation are more likely to develop pulmonary fibrosis. 
• Family history. Some types of pulmonary fibrosis can run in families.

Pulmonary fibrosis symptoms

The most common symptoms or signs of pulmonary fibrosis include:

• Shortness of breath.
• Dry cough.
• Fatigue.
• Unexplained weight loss.
• Pain in your joints or muscles. 
• Tips of the fingers or toes widen.

Some patients develop severe symptoms quickly, while others may develop symptoms progress over time.

Pulmonary fibrosis complications

The most common complications of pulmonary fibrosis include:

• Hypertension in the lungs.
• Blood clots.
• Heart failure in the right ventricle.
• Respiratory failure.
• Lung cancer.
• Depression or anxiety.
• Lung complications, such as collapsed lung or lung infections.

Pulmonary fibrosis diagnosis

Your primary care physician, pulmonologist, or cardiologist can diagnose pulmonary fibrosis. During a clinic exam, your pulmonologist will take a full medical history, discuss your exposure to potential toxins or pollutants, determine if you have other conditions that can affect your lung function, perform a physical exam, and order diagnostic testing.

Imaging tests that may be necessary to diagnose pulmonary fibrosis include:

• Chest X-ray.
• CT scan.
• Echocardiogram.
• Blood tests.

Pulmonary function tests that may be used to diagnose pulmonary fibrosis include:

• Pulmonary function testing.
• Pulse oximetry.
• Exercise stress test.
• Blood gas test.

If imaging or pulmonary function tests have not clearly diagnosed your condition, your doctor may take a lung tissue to examine in a laboratory. A tissue biopsy can diagnose pulmonary fibrosis or rule out other conditions. The biopsy may be performed with a bronchoscopy where your doctor will remove small tissue samples using a bronchoscope that has been passed through your mouth and into the lungs. 

When necessary, your doctor may perform a surgical biopsy to obtain a larger tissue sample. This procedure can be performed minimally invasively or through open surgery. 

Pulmonary fibrosis treatment

There is not a cure for pulmonary fibrosis. The goal of pulmonary fibrosis treatment is to try to slow the progression of the condition and to help you live a healthy, active life.

Treatment for pulmonary fibrosis include:

• Lifestyle modifications. Quitting smoking, eating a healthy diet, and maintaining a healthy weight can help stop pulmonary fibrosis progression.
• Medical management. Your doctor may prescribe medications to slow the disease progression or to relieve symptoms. Your doctor will evaluate your case to determine what medication is most appropriate for your case.
• Oxygen therapy. If you are not able to get enough oxygen into the bloodstream, you may need supplemental oxygen. Oxygen therapy can reduce your shortness of breath and help you stay active.
• Pulmonary rehabilitation. Pulmonary rehab helps patients learn how to exercise, provides nutritional counseling, teaches patients breathing techniques, and provides support to help manage to live with pulmonary fibrosis.
• Lung transplant. Lung transplant is the last resort option for patients with severe pulmonary fibrosis and have failed at all other treatments.

When to Seek Care

If you're experiencing discomfort while breathing, have a dry cough, or are having trouble breathing you should contact your doctor right away to schedule an appointment. If you're experiencing shortness of breath or it's hard to breath, seek immediate medical care.