Idiopathic Pulmonary Fibrosis

Definition

Idiopathic pulmonary fibrosis (IPF) is a chronic disease. It causes inflammation and fibrosis (scarring) of tissue in the lungs. It occurs most often in people aged 50-70 years.There are various types with similar symptoms, but different response to treatment and outcomes.

Causes

Idiopathic means the cause is not known.Researchers think that IPF is an exaggerated and uncontrolled inflammatory response. This produces the scar tissue. What starts the cycle is not known. Over time, scarring surrounds the thin walled air sacs in the lungs. This makes the tissue thicker and stiffer. As a result, breathing becomes difficult. The lungs gradually lose their ability to pass oxygen to the rest of the body.
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Risk Factors

IPF occurs most often in males and people aged 50 years and older. Other factors that may increase your chance of IPF include:
  • Cigarette smoking
  • Viral infection
  • Occupational exposures to dusts containing wood, metal, silica, bacteria, and animal proteins or to aerosol sprays, gases, and fumes
  • Medications such as nitrofurantoin, sulfasalazine, amiodarone, propranolol, methotrexate, cyclophosphamide, bleomycin
  • Gastroesophageal reflux disease (GERD)
  • Other family members with IPF

Symptoms

Over time, the symptoms get worse. This makes daily activities difficult. People with IPF gradually start to have some or all of these symptoms:
  • 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)

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