Pulmonary Fibrosis

• Pulmonary fibrosis refers to scarring of the lung tissue (fibrosis of the lungs).
• Symptoms of pulmonary fibrosis include:
  • Cough
  • Shortness of breath or difficulty breathing
  • Decreased exercise tolerance
• The most common type of pulmonary fibrosis is called idiopathic pulmonary fibrosis; its cause is not understood.
• A number of different medical conditions can also cause pulmonary fibrosis.
• Causes of pulmonary fibrosis can include:
  • Environmental toxins
  • Medications, chronic inflammation
  • Infections
• Treatment options for pulmonary fibrosis are limited as the disease is progressive (worsening over time).
• The prognosis (outlook) is poor for pulmonary fibrosis. The survival (life expectancy) for most patients is less than 5 years.

"Fibrosis" is a term used to refer to scarring, so pulmonary fibrosis means scarring throughout the lungs.

The definition of "idiopathic" is "of unknown cause;" thus idiopathic pulmonary fibrosis (IPF) is fibrosis (scarring) of the lungs without a known cause.

IPF mostly affects middle-aged and older adults, and there is no cure. The progress of the disease varies from person to person, but often people diagnosed with idiopathic pulmonary fibrosis live about three to five years following their diagnosis.

While the cause of IPF is unknown, it is thought that genetics may be a factor. When more than one member of a family has IPF, the disease is referred to as familial idiopathic pulmonary fibrosis.

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)
• 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.