Health Encyclopedia

Idiopathic pulmonary fibrosis

Definition

Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause.

Alternative Names

Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP

Causes, incidence, and risk factors

No one knows what causes pulmonary fibrosis or why some people get it. It causes the lungs to become scarred and stiffened. This stiffening makes it increasingly difficult to breathe. In some people the disease gets worse quickly (over months to a few years), but other people have little worsening of the disease over time.

The condition is believed to result from an inflammatory response to an unknown substance. "Idiopathic" means no cause can be found. The disease occurs most often in people between 50 - 70 years old.

Symptoms

Chest pain (occasionally)
Cough (usually dry)
Decreased tolerance for activity
Shortness of breath during activity that lasts for months or years and over time will also occur at rest

Signs and tests

The health care provider will perform a physical exam and ask questions about your medical history. Your doctor will ask whether you have been exposed to asbestos.

Patients with idiopathic pulmonary fibrosis have abnormal breath sounds called crackles. Patients with advanced disease may have blue-colored skin (cyanosis) around the mouth or in the fingernails due to low oxygen.

Examination of the fingers and toes may show abnormal enlargement of the fingernail bases (clubbing).

Tests that help diagnose idiopathic pulmonary fibrosis include the following:

Bronchoscopy with transbronchial lung biopsy
Chest CT scan
Chest x-ray
Measurements of blood oxygen level
Pulmonary function tests
Surgical lung biopsy
Tests for connective tissue diseases such as rheumatoid arthritis, lupus, or scleroderma

Treatment

No known cure exists for idiopathic pulmonary fibrosis. Medications such as corticosteroids and cytotoxic drugs may be given to reduce swelling (inflammation), but these treatments usually don't work. Oxygen is given to patients who have low blood oxygen levels.

Some patients with advanced pulmonary fibrosis may need a lung transplant. Lung rehabilitation will not cure the lung disease, but it can help maintain exercise capacity.

Support Groups

You can ease the stress of illness by joining a support group where members share common experiences and problems.

Expectations (prognosis)

Some patients may improve when they are treated with corticosteroids or cytotoxic drugs, but in most people the disease can get worse even with treatment. This worsening can happen quickly, or very slowly.

Complications

Chronic hypoxemia (low blood oxygen level)
Cor pulmonale
Pneumothorax
Polycythemia (abnormally high levels of red blood cells)
Pulmonary hypertension
Respiratory failure

Calling your health care provider

Call for an appointment with your health care provider if you develop a regular cough or shortness of breath.

Prevention

Avoiding smoking may help prevent this condition, but how to prevent the cause is not known.

References

Goldman L, Ausiello D. Goldman: Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders; 2007.

Review Date:1/18/2008
Reviewed By:Andrew Schriber, MD, FCCP, Specialist in Pulmonary, Critical Care, and Sleep Medicine, Virtua Memorial Hospital, Mount Holly, New Jersey. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

ADAM Quality A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The Agency for Health Care Administration (Agency) and this website do not claim the information on, or referred to by, this site is error free. This site may include links to websites of other government agencies or private groups. Our Agency and this website do not control such sites and are not responsible for their content. Reference to or links to any other group, product, service, or information does not mean our Agency or this website approves of that group, product, service, or information.

Additionally, while health information provided through this website may be a valuable resource for the public, it is not designed to offer medical advice. Talk with your doctor about medical care questions you may have.