Idiopathic pulmonary fibrosis
Definition
Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause.
Alternative Names
Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIPCauses
No one knows what causes pulmonary fibrosis or why some people get it. It causes the lungs to become scarred and stiffened. This stiffening makes it increasingly difficult to breathe. In some people the disease gets worse quickly (over months to a few years), but other people have little worsening of the disease over time.
The condition is believed to result from an inflammatory response to an unknown substance. "Idiopathic" means no cause can be found. The disease occurs most often in people between 50 - 70 years old.
Symptoms
- Chest pain (occasionally)
- Cough (usually dry)
- Decreased tolerance for activity
- Shortness of breath during activity that lasts for months or years and over time will also occur at rest
Exams and Tests
The health care provider will perform a physical exam and ask questions about your medical history. Your doctor will ask whether you have been exposed to asbestos.
Patients with idiopathic pulmonary fibrosis have abnormal breath sounds called crackles. Patients with advanced disease may have blue-colored skin (cyanosis) around the mouth or in the fingernails due to low oxygen.
Examination of the fingers and toes may show abnormal enlargement of the fingernail bases (clubbing).
Tests that help diagnose idiopathic pulmonary fibrosis include the following:
- Bronchoscopy with transbronchial lung biopsy
- Chest CT scan
- Chest x-ray
- Measurements of blood oxygen level
- Pulmonary function tests
- Surgical lung biopsy
- Tests for connective tissue diseases such as rheumatoid arthritis, lupus, or scleroderma
Treatment
No known cure exists for idiopathic pulmonary fibrosis. Medications such as corticosteroids and cytotoxic drugs may be given to reduce swelling (inflammation), but these treatments usually don't work. Oxygen is given to patients who have low blood oxygen levels.
Some patients with advanced pulmonary fibrosis may need a lung transplant. Lung rehabilitation will not cure the lung disease, but it can help maintain exercise capacity.
Support Groups
You can ease the stress of illness by joining a support group where members share common experiences and problems.
See also: Lung disease - support group
Outlook (Prognosis)
Some patients may improve when they are treated with corticosteroids or cytotoxic drugs, but in most people the disease can get worse even with treatment. This worsening can happen quickly, or very slowly.
Possible Complications
- Chronic hypoxemia (low blood oxygen level)
- Cor pulmonale
- Pneumothorax
- Polycythemia (abnormally high levels of red blood cells)
- Pulmonary hypertension
- Respiratory failure
When to Contact a Medical Professional
Call for an appointment with your health care provider if you develop a regular cough or shortness of breath.
Prevention
Avoiding smoking may help prevent this condition, but how to prevent the cause is not known.
References
Goldman L, Ausiello D. Goldman: Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders; 2007.Reviewed By: Andrew Schriber, MD, FCCP, Specialist in Pulmonary, Critical Care, and Sleep Medicine, Virtua Memorial Hospital, Mount Holly, New Jersey. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
