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Pulmonary Fibrosis

Pulmonary fibrosis is a disease in which tissue deep in the lungs becomes thick and stiff, or scarred, over time. The formation of scar tissue is called fibrosis. As the lung tissue thickens, the lungs can't properly move oxygen into the bloodstream. As a result, the brain and other organs do not get the oxygen they need.

In the majority of cases, doctors cannot find a cause for the illnesses which is why it is called idiopathic pulmonary fibrosis. Genetics can play an important role in the development of IPF and there is a greater risk of developing the disease if a family member has or has had the disease (familial IPF).

Idiopathic pulmonary fibrosis is a serious disorder that usually affects middle-aged and older adults and varies from person to person. In some cases, the disease stays the same for years and in others, the fibrosis happens quickly posing a risk of death. IPF can increase the risk of having other complications, some of which are very serious such as respiratory insufficiency (inadequate oxygenation of the blood), pulmonary hypertension, cardiac failure, pulmonary embolism, pneumonia or lung cancer.

Symptoms

The signs and symptoms of idiopathic pulmonary fibrosis (IPF) develop over time. They may not even begin to appear until the disease has done serious damage to your lungs.

The most common signs and symptoms are:

  • Shortness of breath. This usually is the main symptom of IPF. At first, you may be short of breath only during exercise. Over time, you'll likely feel breathless even at rest.
  • A dry, hacking cough that doesn't get better. Over time, you may have repeated bouts of coughing that you can't control.
  • Rapid, shallow breathing
  • Gradual, unintended weight loss
  • Fatigue (tiredness) or malaise (a general feeling of being unwell)
  • Aching muscles and joints
  • Clubbing, which is the widening and rounding of the tips of the fingers or toes

Prevention

Given that the causes nor the triggers of this disease are known, there are no effective measures that can be taken to prevent its development. Nonetheless, general measures (quit smoking, regular physical exercise) can be taken to have a better physical condition and to better manage the symptoms that do appear. Since the causes of pulmonary fibrosis can also be job-related, it is particularly important for workers to take precautions.

Gene or region studied

  • DSP
  • TERT
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