Definition

Idiopathic pulmonary fibrosis (IPF) is a chronic disease of the lungs. It causes inflammation and scarring of lung tissue.

Causes

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.

Healthy Air Sacs of Lungs Copyright © Nucleus Medical Media, Inc.

Risk Factors

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

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
• Fatigue
• Enlargement of the fingertips, called clubbing, or sometimes the toes

Diagnosis

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:

• Chest x-ray
• CT scan
• Bronchoscopy

Your lung function may be tested. This can be done with:

• Pulmonary function tests
• An exercise test

Treatment

There is no known cure. The goal of treatment is to ease symptoms. Options include:

Prevention

There is no proven way to prevent most IPF. Proper protection when working at a high risk job may help.