Idiopathic Pulmonary Fibrosis

ιδιοπαθής πνευμονική ίνωσηWhat is idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis (IPF = Idiopathic pulmonary fibrosis) is a lung condition that causes difficulty in breathing. Τhe airspaces of the lungs, where the transport of oxygen to the blood takes place, get destroyed. Finally, scars are created at the lungs. These changes result in patients complaining of dyspnea and cough. Usually, patients are older than 40 years. This is a serious illness which can not be cured and instead deteriorates over time.

What is the cause of idiopathic pulmonary fibrosis?

Doctors do not know exactly the causes leading someone to idiopathic pulmonary fibrosis. However, risk factors have been found and are the following:

  • Smoking
  • Inhalation of toxic chemicals and high air pollution
  • Inhalation of microscopic materials eg dust, for a long time
  • Family history of idiopathic pulmonary fibrosis.

What are the symptoms of idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis may have a slow course and is completely asymptomatic in early stages. However, when symptoms occur, these may include:

  • Shortness of breath especially when exercising
  • Dry cough

Are there any tests to be done for the diagnosis of idiopathic pulmonary fibrosis?

Yes, your doctor may recommend the following:

  1. Blood tests to exclude other lung diseases. Unfortunately, there is not any blood test that is specific for idiopathic pulmonary fibrosis.
  2. Breath tests. With these, your doctor may discriminate whether your symptoms are caused by idiopathic pulmonary fibrosis, or another lung disease, eg COPD.
  3. High resolution CT (HRCT). It depicts in great detail the inside of the human body.
  4. If your doctor is not sure whether you have idiopathic pulmonary fibrosis, he may recommend that you have a lung biopsy. With this procedure,  a small piece of lung is biopsied and examined under the microscope.

What is the treatment of idiopathic pulmonary fibrosis?

Unfortunately there is no treatment that cures idiopathic pulmonary fibrosis. Usually the disease worsens slowly over time. Fortunately, during the last years, there have been produced new antifibrotic medicines that could slow the deterioration rate of the disease. Moreover, your doctor may recommend you some measures to treat your symptoms. These usually include the following:

  • Smoking cessation. It is necessary to stop smoking because smoking further deteriorates lung function.
  • Oxygen. As the disease worsens over time, oxygen may be needed due to respiratory failure
  • Pulmonary rehabilitation. In a rehabilitation program, patients learn some breathing techniques, train to deal with their symptoms and train with special muscle exercises.
  • Treatment of gastroesophageal reflux (GERD). In GERD, the acid from the stomach goes up into the esophagus and this was found to worsen idiopathic pulmonary fibrosis.
  • Special medication. Antifibrotics. There are some promising new drugs that may help patients with idiopathic pulmonary fibrosis, as some studies have already shown.
  • Lung transplantation. With this surgical procedure, one or both lungs are replaced by donor’s normal lungs. Certain criteria must be met by the candidate.

Sometimes idiopathic pulmonary fibrosis has a very rapid deterioration, a situation which is called an exacerbation. If this happens, your doctor may prescribe antibiotics and cortisone. Also, annual vaccination against influenza and pneumococcal vaccination at least once in lifetime are recommended.

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