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Idiopathic Pulmonary Fibrosis

Definition:
Idiopathic pulmonary fibrosis (IPF) is a chronic disease that causes inflammation (swelling and irritation) and fibrosis (scarring) of tissue in the lungs. It usually occurs in people between ages 50 and 70.

Causes:
The word “idiopathic” in the name of this disease means the cause is not known. Researchers think that IPF involves an exaggerated and uncontrolled inflammatory response that produces the fibrous scar tissue. What starts the injury and abnormal healing is not known. Over time, the scarring surrounds the thin-walled small air sacs in the lungs (alveoli). This makes the tissues become thicker and stiffer. As a result, breathing becomes difficult, and the lungs gradually lose their ability to provide oxygen to the rest of the body.

Risk Factors:
A risk factor is something that increases your chance of getting a disease or condition. Although the cause of IPF is not known, the factors below may increase the risk of getting it:
  • Cigarette smoking
  • Occupational exposure to metal or wood dust
  • Viral infection
  • Other family members with IPF
Symptoms:
  • People with IPF gradually start to have some or all of the symptoms below. Over time, the symptoms get worse, making daily activities difficult.
  • Shortness of breath, at first only during or after physical activity, but later also when resting
  • Dry cough
  • Gradual weight loss
  • Fatigue
  • Clubbing (enlargement of the fingertips or sometimes the toes)
Diagnosis:
The doctor will ask about your symptoms and medical history and perform a physical exam. One or more of the following tests may be performed:
  • Chest X-ray or CT scan (to view the lungs and check for scarring)
  • Pulmonary Function Tests (breathing tests to measure the size and effectiveness of the lungs)
  • Blood test (measurement of oxygen level in the blood to tell how well the lungs are taking up oxygen)
  • Exercise Test (on treadmill or stationary bicycle to measure how well the lungs and heart work during physical activity)
  • Bronchoalveolar Lavage (Fluid is put into the airways through a tube in the nose and mouth and then removed to study the cells and check for signs of inflammation.)
  • Lung Biopsy (a surgical procedure in which a small sample of lung tissue is removed and studied. This is usually required to confirm a diagnosis of IPF.
Treatment:
IPF worsens over time, and there is no known cure. The goal of treatment is to improve symptoms and slow the disease process by reducing inflammation and scarring. The tissue that is already scarred cannot be returned to normal.

Medication is the main form of treatment, although it does not work for some people. A common combination is prednisone to reduce inflammation along with cytoxan to reduce the body’s immune response. Some people may need to receive oxygen to help them breathe. Maintaining a healthy lifestyle in term of diet, exercise, rest, and not smoking may also help slow the disease. Lung transplantation may be considered for people with advanced IPF who do not respond to other treatment.

Prevention:
There is no proven way to prevent IPF. However, avoiding smoking may help.
 
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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