Pulmonary Fibrosis
 

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What is Pulmonary Fibrosis?

Pulmonary fibrosis is a progressive scarring of the lungs that increasingly interferes with a patient's ability to breathe. Idiopathic means "of unknown cause" thus, idiopathic pulmonary fibrosis is pulmonary fibrosis that cannot be positively traced to a particular cause. This is the case in most pulmonary fibrosis patients.

Scarring on the lungs occurs when the lungs' normal healing process is disrupted. Whatever the cause, the lungs' air sacs are gradually replaced by fibrous tissue. As the cycle of injury and lung response continues, this tissue becomes thicker and stiffer, causing a loss of the tissue's ability to transfer oxygen into the bloodstream.

Causes

In cases of pulmonary fibrosis some known causes include:

  • Exposure to metal or wood dust, gases or fumes
  • Sarcoidosis
  • Some medications
  • Diseases of connective tissue, including rheumatoid arthritis and systemic sclerosis

Some risk factors associated with IPF include:

  • Cigarette smoking
  • Viral infection
  • Radiation
  • More rarely, there is sometimes a familial link with IPF

Symptoms

  • Spasmodic, dry cough
  • Shortness of breath, particularly with physical activity
  • Fatigue
  • Weight loss

Progression

Pulmonary fibrosis is generally a progressive disease that tends to worsen over time. The rate of degeneration varies between patients depending upon the age at which it is contracted, health of the patient and response to treatment. Some patients are able to maintain a normal lifestyle for years. However, pulmonary fibrosis can be disabling and even fatal.

Diagnosis

The scarring and symptoms of IPF can be similar to other interstitial lung diseases (ILDs), making it difficult to diagnose. These 200 or so disorders share the characteristic of scarring on the interstitium tissue between air sacs in the lungs. Thus, diagnosing IPF includes ruling out many other ILDs.

New standards and improved test procedures are making diagnosis of IPF more accurate. At MPC, we do the following to assess your condition:

  • Thorough physical exam
  • Obtain a complete medical history
  • Pulmonary function tests
  • Blood tests
  • Chest x-ray or CT scan

If the evidence continues to point to IPF, we may then perform a lung biopsy for a final determination.

Prevalence

Because IPF is difficult to diagnose, estimates of the number of cases vary widely - from 50,000 to 200,000 in the U.S. Even at the high estimate, that makes this condition fairly rare. More men than women contract IPF, and it is typically diagnosed between the ages of 50 and 70.

Treatment

Currently, treatments for IPF can improve symptoms and may slow the progression of the disease. As yet there is no known cure. Standard treatments are:

  • Use of an anti-inflammatory corticosteroid
  • Suppression of the body's immune system with pharmaceuticals

As the disease becomes more serious, supplemental oxygen therapy may be required. In some cases, when impairment is severe, lung transplant may be considered.

Patient Actions

  • If you smoke, begin a cessation program.
  • Take prescribed medication as instructed.
  • Eat a balanced diet to maintain strength.
  • Eat smaller meals more frequently. Patients often find that breathing comes easier when their stomachs are not full.
  • Exercise moderately to maintain strength and lung function. Talk to your doctor about a program that fits your condition.
  • Enroll in a pulmonary rehabilitation program to learn breathing techniques and expand your support network.
  • Join a support group.

    More Information

  • www.coalitionforpf.org This is the Coalition for Pulmonary Fibrosis's site which contains a wealth of detail.
  • www.lungusa.org Click on Diseases A to Z in the top navigation, then scroll down to find Interstitial Lung Disease and Pulmonary Fibrosis under "I".
  • www.pulmonaryfibrosis.org. This is the Pulmonary Fibrosis Foundation's site which includes information and links to learn more about current research, education and advocacy on IPF.