Idiopathic pulmonary fibrosis
St. Luke's Hospital
Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
Find a Physician Payment Options Locations & Directions
Follow us on: facebook twitter Mobile Email Page Email Page Print Page Print Page Increase Font Size Decrease Font Size Font Size
America's 50 Best Hospitals
Meet the Doctor
Spirit of Women
Community Health Needs Assessment
Home > Health Information

Multimedia 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

No one knows what causes idiopathic pulmonary fibrosis or why some people get it. It causes the lungs to become scarred and stiffened. This stiffening may make 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 be due to an inflammatory response to an unknown substance or injury. "Idiopathic" means no cause can be found. The disease occurs most often in people between 50 and 70 years old.

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 questions about your medical history. Your doctor will ask whether you have been exposed to asbestos and if you have been a smoker.

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

No known cure exists for idiopathic pulmonary fibrosis. Unfortunately, no medication has been shown to improve the outcome of patients with this condition.

  • For some people, medications such as corticosteroids and cytotoxic drugs may help reduce swelling (inflammation). However, these drugs might also increase the risk of death.
  • Other new treatments that have been shown to help some people with idiopathic pulmonary fibrosis are being studied.
  • Patients with low blood oxygen levels may need oxygen.
  • Lung rehabilitation will not cure the disease, but it can help maintain exercise capacity (the ability to exercise without breathing difficulty).

You can make home and lifestyle changes to manage breathing symptoms. Anyone who smokes should 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 where members share common experiences and problems.

The following organizations are good resources for information on lung disease:

Outlook (Prognosis)

Some patients may improve or stay stable for a long time when they are treated with corticosteroids or cytotoxic drugs. However, in most people the disease can get worse even with treatment. This worsening can happen quickly, or very slowly.

When breathing symptoms become more severe, discuss treatments that prolong life, health care agents, and advanced care directives with your health care provider.

Possible Complications

When to Contact a Medical Professional

Call your health care provider if your breathing is:

  • Getting harder
  • Faster than before
  • Shallow, and you cannot get a deep breath

Also call your health care provider if:

  • You need to lean forward when sitting to breathe comfortably.
  • You are using muscles around your ribs to help you breathe.
  • You are having headaches more often.
  • You feel sleepy or confused.
  • You have a fever.
  • You are coughing up dark mucus.
  • Your 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.

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: Saunders Elsevier; 2010:chap 57.


Review Date: 5/29/2012
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.
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.
adam.com
 


Back  |  Top
About Us
Contact Us
History
Mission
Locations & Directions
Quality Reports
Annual Reports
Honors & Awards
Community Health Needs
Assessment

Newsroom
Services
Brain & Spine
Cancer
Heart
Maternity
Orthopedics
Pulmonary
Sleep Medicine
Urgent Care
Women's Services
All Services
Patients & Visitors
Locations & Directions
Find a Physician
Tour St. Luke's
Patient & Visitor Information
Contact Us
Payment Options
Financial Assistance
Send a Card
Mammogram Appointments
Health Tools
My Personal Health
mystlukes
Spirit of Women
Health Information & Tools
Clinical Trials
Health Risk Assessments
Employer Programs -
Passport to Wellness

Classes & Events
Classes & Events
Spirit of Women
Donate & Volunteer
Giving Opportunities
Volunteer
Physicians & Employees
For Physicians
Remote Access
Medical Residency Information
Pharmacy Residency Information
Physician CPOE Training
Careers
Careers
St. Luke's Hospital - 232 South Woods Mill Road - Chesterfield, MO 63017 Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
Copyright © St. Luke's Hospital Website Terms and Conditions  |  Privacy Policy  |  Patient Notice of Privacy Policies PDF Sitemap St. Luke's Mobile