Saturday, February, 11, 2012

Idiopathic pulmonary fibrosis

Table of Contents

Alternative Names

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


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).
  • Ongoing clinical studies are experimenting with new treatments that have been shown to help some people with idiopathic pulmonary fibrosis.
  • Oxygen may be necessary in patients who have low blood oxygen levels.
  • Lung rehabilitation will not cure the disease, but can help maintain exercise capacity (the ability to exercise without breathing difficulty).

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.

See also: Lung disease - support group


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
  • Shortness of breath

Review Date: 04/27/2010
Reviewed By: Allen J. Blaivas, DO, Clinical Assistant Professor of Medicine UMDNJ-NJMS, Attending Physician in the Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Veterans Affairs, VA New Jersey Health Care System, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)