Idiopathic pulmonary fibrosis

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Clubbing

Respiratory system

Spirometry

Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP

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.

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.

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.

• Respiratory failure
• Chronic hypoxemia (low blood oxygen level)
• Pulmonary hypertension
• Cor pulmonale (failure of the right side of the heart)
• Polycythemia (abnormally high levels of red blood cells)

Call for an appointment with the health care provider if persistent cough or shortness of breath develops.

Review Date: 05/03/2006
Reviewed By: David A. Kaufman, M.D., Assistant Professor, Division of Pulmonary, Critical Care & Sleep Medicine, Mount Sinai School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.