Idiopathic pulmonary fibrosis involves scarring or thickening of tissues deep in the lung without a known cause.
Alternative Names
Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP
Causes, incidence, and risk factors
Idiopathic pulmonary fibrosis is a disease of the lower respiratory tract that damages the air sacs (alveoli) and leads to reduced transfer of oxygen to the blood. It causes widespread scarring of the lung. The condition is believed to result from an inflammatory response to an unknown agent -- "idiopathic" means no cause can be found. The disease occurs most often in people between 50 and 70 years old. Nearly 75% of patients with idiopathic pulmonary fibrosis have smoked cigarettes.
Signs and tests
In addition to a physical examination , your health care provider will take a careful history in order to exlude other similar diseases. Patients with idiopathic pulmonary fibrosis have dry, Velcro-like breath sounds called crackles. Patients with advanced disease may have cyanosis (blueness around the mouth or in the fingernails due to low oxygen). Examination of the fingers and toes may reveal clubbing (abnormal enlargement of the tips).
Tests that help diagnose idiopathic pulmonary fibrosis include the following:
Chest X-ray High-resolution CT scan of the chest
Pulmonary function tests Bronchoscopy with transbronchial lung biopsy Surgical lung biopsy
Measurement of blood oxygen level at rest or during exertion 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 suppress inflammation, but these treatments are usually unsuccessful. Oxygen is given to patients who have low blood oxygen levels. Recently, interferon-gamma-1B has shown some promise in treating this disease, but more research is necessary to demonstrate that this drug is beneficial.
Lung transplantation may be indicated for some patients with advanced pulmonary fibrosis.
Support groups
The stress of illness can often be helped by joining a support group where members share common experiences and problems. See lung disease - support group .
Expectations (prognosis)
Some patients may improve on treatment with corticosteroids or cytotoxic drugs, but most patients suffer from progressive disease despite treatment. Pulmonary hypertension (high blood pressures in the vessels of the lungs) and respiratory failure is the eventual outcome. Average survival time is 5-6 years, but this varies greatly between patients.
Calling your health care provider
Call for an appointment with the health care provider if persistent cough or shortness of breath develops.
Prevention
Avoiding smoking may help prevent this condition but its cause, and therefore more specific prevention, is not known.