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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

Doctors do not know what causes idiopathic pulmonary fibrosis (IPF) or why some people get it. Idiopathic means the cause is not known. The condition may be due to the lungs responding to an unknown substance or injury. Genes may play a role in developing IPF. The disease occurs most often in people between 50 and 70 years old.

When you have IPF, your lungs become scarred and stiffened. This makes it hard for you to breathe. In some people, IPF gets worse quickly over months or a few years.  In others, IPF worsens over a much longer time.

Symptoms

  • Chest pain (occasionally)
  • Cough (usually dry)
  • Decreased tolerance for activity
  • Shortness of breath during activity (this symptom lasts for months or years, and over time may also occur when at rest)

Exams and Tests

The health care provider will do a physical exam and ask about your medical history. You will be asked whether you have been exposed to asbestos or other toxins and if you have been a smoker.

The physical exam may find that you have:

  • Abnormal breath sounds called crackles
  • Blue-colored skin (cyanosis) around the mouth or fingernails due to low oxygen (with advanced disease)
  • Enlargement of the fingernail bases, called clubbing (with advanced disease)

Tests that help diagnose idiopathic pulmonary fibrosis include the following:

Treatment

There is no known cure for IPF.

Treatment is aimed at relieving symptoms:

  • Medicines such as corticosteroids may help reduce swelling (inflammation) inside the lungs.
  • Patients with low blood oxygen levels may need oxygen support at home.
  • Lung rehabilitation will not cure the disease, but it can help people exercise without difficulty breathing.

Making home and lifestyle changes can help manage breathing symptoms. If you or any family members smoke, now is the time to stop.

Some patients with advanced pulmonary fibrosis may need a lung transplant.

Medicines are now being tested to see if they can slow the disease.

Support Groups

You can ease the stress of illness by joining a support group. Sharing with others who have common experiences and problems can help you not feel alone.

These organizations are good resources for information on lung disease:

Outlook (Prognosis)

Some patients may improve or stay stable for a long time with or without treatment. Most people get worse, even with treatment.

When breathing symptoms become more severe, you and your doctor should discuss treatments that prolong life, such as lung transplantation. Also discuss advance care planning.

Complications of IPF may include:

  • Abnormally high levels of red blood cells due to low blood oxygen levels
  • Collapsed lung
  • High blood pressure in the arteries of the lungs
  • Respiratory failure

When to Contact a Medical Professional

Call your health care provider right away if you have any of the followinig:

  • Breathing that is harder, faster, or shallower (you are unable to take a deep breath)
  • To lean forward when sitting to breathe comfortably
  • Frequent headaches
  • Sleepiness or confusion
  • Fever
  • Dark mucus when you cough
  • Blue fingertips or skin around your fingernails

References

American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), and Latin American Thoracic Association (ALAT). Idiopathic pulmonary fibrosis: evidence based guideline for diagnosis and management. Am J Respir Crit Care Med. 2011; 183:788-824.

Raghu G. Interstitial lung disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 92.

Selman M, Morrison LD, Noble PW, King TE Jr. Idiopathic interstitial pneumonias. In: Mason RJ, Broaddus VC, Martin TR, et al., eds. Murray and Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa: Elsevier Saunders; 2010:chap 57.


Review Date: 4/26/2014
Reviewed By: Denis Hadjiliadis, MD, Assistant Professor of Medicine, Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pa. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial Team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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