Navigate Up

Women's Center - A-Z Index

#
Y

Print This Page

Idiopathic pulmonary fibrosis

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 perform a physical exam and ask about your medical history. You will be asked whether you have been exposed to asbestos 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)

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:

Treatment

There is no known cure exists 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 maintain the ability to exercise without breathing difficulty.

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

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

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 advanced care planning .

Possible Complications

When to Contact a Medical Professional

Call your health care provider promptly if you have any of the following::

  • Your breathing is getting harder, faster, or shallow (are unable to take a deep breath)
  • Need to lean forward when sitting to breathe comfortably.
  • Frequent headaches
  • Feel sleepy or confused
  • Fever
  • Coughing up dark mucus
  • Fingertips or the skin around your fingernails is blue

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.

Updated: 6/3/2013

Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.


©  UPMC | Affiliated with the University of Pittsburgh Schools of the Health Sciences
Supplemental content provided by A.D.A.M. Health Solutions. All rights reserved.

For help in finding a doctor or health service that suits your needs, call the UPMC Referral Service at 412-647-UPMC (8762) or 1-800-533-UPMC (8762). Select option 1.

UPMC is an equal opportunity employer. UPMC policy prohibits discrimination or harassment on the basis of race, color, religion, ancestry, national origin, age, sex, genetics, sexual orientation, marital status, familial status, disability, veteran status, or any other legally protected group status. Further, UPMC will continue to support and promote equal employment opportunity, human dignity, and racial, ethnic, and cultural diversity. This policy applies to admissions, employment, and access to and treatment in UPMC programs and activities. This commitment is made by UPMC in accordance with federal, state, and/or local laws and regulations.

Medical information made available on UPMC.com is not intended to be used as a substitute for professional medical advice, diagnosis, or treatment. You should not rely entirely on this information for your health care needs. Ask your own doctor or health care provider any specific medical questions that you have. Further, UPMC.com is not a tool to be used in the case of an emergency. If an emergency arises, you should seek appropriate emergency medical services.

For UPMC Mercy Patients: As a Catholic hospital, UPMC Mercy abides by the Ethical and Religious Directives for Catholic Health Care Services, as determined by the United States Conference of Catholic Bishops. As such, UPMC Mercy neither endorses nor provides medical practices and/or procedures that contradict the moral teachings of the Roman Catholic Church.

© UPMC
Pittsburgh, PA, USA UPMC.com