Idiopathic pulmonary fibrosis
Also called: IPF, Interstitial lung disease
Progressive scarring of the lung tissue for no known reason. The lungs become stiff, oxygen transfer falls and breathlessness steadily worsens.
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Causes
- Unknown; abnormal repair after repeated injury to the alveolar lining
Risk factors
- Age over 60
- Male sex
- Smoking
- Occupational dusts
- Gastro-oesophageal reflux
- Family history (telomere and MUC5B genes)
Symptoms
- Gradually worsening breathlessness
- Dry cough
- Finger clubbing
- Fine crackles at the lung bases
Diagnosis — tests and examinations
- High-resolution CT (usual interstitial pneumonia pattern)
- Lung function tests (restrictive pattern, low gas transfer)
- Multidisciplinary discussion; biopsy occasionally
Treatment
Medicines and medical treatment
- Antifibrotics: pirfenidone or nintedanib slow decline
- Oxygen therapy
- Treatment of reflux and cough
Operations and procedures
- Lung transplantation for suitable patients
Self-care, home remedies and lifestyle
- Pulmonary rehabilitation
- Stop smoking
- Vaccinations
- Early palliative support for breathlessness
Possible complications
- Respiratory failure
- Acute exacerbations
- Pulmonary hypertension
- Lung cancer
Prevention
- Avoid smoking and occupational dust
Outlook
Median survival is 3–5 years from diagnosis, although antifibrotic drugs slow progression.
When to see a doctor
See a doctor for breathlessness or dry cough that persists for more than a few weeks.
Sources and further reading
These sources cover this condition. They have not been checked against every statement on this page.
Related conditions
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