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November 6, 2011
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Pulmonary Fibrosis

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What is pulmonary fibrosis?

"Fibrosis" is a term used to refer to scarring, so pulmonary fibrosis means scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes (sarcoidosis, Wegener's granulomatosis ), infections, environmental agents (asbestos, silica, exposure to certain gases), exposure to ionizing radiation (such as radiation therapy to treat tumors of the chest), chronic conditions (lupus, rheumatoid arthritis), and certain medications.

In a condition known as hypersensitivity pneumonitis, fibrosis of the lung can develop following a heightened immune reaction to inhaled organic dusts or occupational chemicals. This condition most often results from inhaling dust contaminated with bacterial, fungal, or animal products.

In some people, chronic pulmonary inflammation and fibrosis develop without an identifiable cause. Most of these people have a condition called idiopathic pulmonary fibrosis (IPF) that does not respond to medical therapy, while some of the other types of fibrosis, such as nonspecific interstitial pneumonitis (NSIP), may respond to immune suppressive therapy.

Synonyms (other names) for various types of pulmonary fibrosis that have been used in the past include chronic interstitial pneumonitis, Hamman-Rich syndrome, and diffuse fibrosing alveolitis.

What are the causes and symptoms of pulmonary fibrosis?

Symptoms of pulmonary fibrosis include:

Symptoms vary depending on the cause of the pulmonary fibrosis. The severity of symptoms and the progression (worsening) of symptoms over time can vary.

The most common cause, idiopathic pulmonary fibrosis, unfortunately often has slow and relentless progression. Early on, patients often complain of a dry unexplained cough. Often, slow and insidious onset of shortness of breath can set in. With time, dyspnea (shortness of breath) worsens. Primarily, dyspnea initially occurs only with activity and is often attributed to aging. With time, the dyspnea begins to occur with less and less activity. Eventually, the shortness of breath becomes disabling, limiting all activity and even occurring while sitting still. In rarer cases, the fibrosis can be rapidly progressive, with dyspnea and disability occurring in weeks to months of onset of the disease. This form of pulmonary fibrosis has been referred to as Hamman-Rich syndrome.



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Pulmonary Fibrosis

What is hypersensitivity pneumonitis and what causes it?

Hypersensitivity pneumonitis is an inflammation of the lung (usually of the very small airways) caused by the body's immune reaction to small air-borne particles. These particles can be bacteria, mold, fungi, or even inorganic matter.

What is acute hypersensitivity pneumonitis?

Acute hypersensitivity pneumonitis tends to occur 4-12 hours after exposure (usually heavy exposure) to the particles.

What are the symptoms of acute hypersensitivity pneumonitis?

Symptoms of acute hypersensitivity pneumonitis include:

  • fever,
  • chills,
  • coughing,
  • shortness of breath,
  • body aches, etc.

Chest x-rays may show diffuse small nodules in the lungs. Typically, the symptoms will subside hours to days after exposure (provided there are no repeated exposures). The Chest x-ray abnormalities w...

Read the Hypersensitivity Pneumonitis article »







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