Idiopathic Pulmonary Fibrosis Overview

Idiopathic Pulmonary Fibrosis Overview
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Idiopathic pulmonary fibrosis is a condition in which the lungs become scarred and thickened without any apparent cause. When the lungs become thick and stiff, the patient has an increasingly hard time breathing. People between the ages of 50 and 70 are most likely to develop this disease.

Signs and Symptoms

The severity of signs and symptoms is very individualized and varies greatly. Shortness of breath and a dry cough are often the first symptoms a patient experiences. In addition to these, patients may experience:

  • Fatigue
  • Achy joints and muscles
  • Unexplained weight loss
  • Occasional chest pain
  • Reduced tolerance for activity

Certain complications may also occur. These may include:

  • Low blood-oxygen levels
  • Right-side heart failure
  • Pulmonary hypertension
  • Respiratory failure

Risk Factors

Though the exact cause of idiopathic pulmonary fibrosis is not known, certain risk factors have been identified. These may include:

  • Being in the middle to older adult years
  • Environmental and occupational toxin exposure
  • Being male
  • Chemotherapy and radiation therapy
  • Genetics
  • Smoking
  • Certain viruses, such as Epstein-Barr

Diagnosis

The patient’s doctor will begin by fully examining them and asking them to describe their symptoms. To come to a definitive diagnosis the patient’s doctor may order the following tests:

  • Chest x-ray: This will typically show if the patient’s lungs have any scar tissue
  • Pulmonary functioning testing: This will show how well the patient’s lungs are working overall
  • Exercise stress test: This will show how the patient’s lungs are functioning while active
  • High-resolution computerized tomography scan: This test will provide the doctor with detailed images of the patient’s lungs
  • Oximetry: This test is simple and will show the patient’s blood-oxygen saturation
  • Bronchoscopy: This is a procedure used to remove a small tissue sample so that it can be further analyzed
  • Surgical biopsy: This is a procedure that is invasive and is done to obtain a larger tissue sample to be further analyzed
  • Bronchoalveolar lavage: This procedure is done to obtain air sac cells for further evaluation

Treatment

As of today, there is no cure for this condition. There are treatments available to help control symptoms, but these treatments are not effective for all patients. Treatments include:

  • Cytotoxic drugs and corticosteroids to help reduce inflammation. An immunosuppressant medication may also be prescribed, as well as N-acetylcysteine. Clinical trials are currently being conducted to determine the effectiveness of pirfenidone in improving lung function and preventing lung tissue destruction
  • Oxygen to increase the patient’s blood-oxygen levels. This may also help to reduce blood pressure and lower a patient’s risk of developing right-side heart failure
  • Lung rehabilitation may be done in an attempt to help patients live satisfying, full lives. This treatment focuses on learning to breathe more efficiently, emotional support, exercise, learning more about this condition, and nutritional counseling
  • Lung transplant when this condition is advanced

Resources

MedlinePlus. (2010). Idiopathic Pulmonary Fibrosis. Retrieved on October 10, 2010 from MedlinePlus: https://www.nlm.nih.gov/medlineplus/ency/article/000069.htm

MayoClinic.com. (2009). Pulmonary Fibrosis. Retrieved on October 9, 2010 from MayoClinic.com: https://www.mayoclinic.com/health/pulmonary-fibrosis/DS00927