Idiopathic pulmonary fibrosis

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Spirometry

Sarcoid, stage IV - chest X-ray

Idiopathic pulmonary fibrosis

Alternative Names:

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

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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.

Complications: