Idiopathic pulmonary fibrosis
Definition
Idiopathic Pulmonary Fibrosis (IPF) is a chronic and progressive lung disease characterised by the scarring of lung tissue. The term "idiopathic" means the cause is unknown, making IPF a diagnosis of exclusion. It mainly affects the interstitial spaces of the lungs, reducing their ability to expand and contract properly.
Aetiology
The exact cause is unknown. It could be linked to:
- Genetic susceptibility
- Environmental exposures such as cigarette smoke and occupational hazards
- Viral infections
- An abnormal immune response
Pathophysiology
There is an overproduction of collagen in the lung tissue. This extra collagen interferes with the lung's gas exchange ability, causing a gradual decline in lung function over time.
Risk factor
- Age greater than 50 years
- Men are more at risk
- Smoking
- Work-related exposures such as asbestos or wood dust
- Family history of pulmonary fibrosis
Sign and Symptoms
- Persistent non-productive cough
- Shortness of breath on exertion (SOBOE)
- Tiredness
- Weight loss
- Chest discomfort or chest pain
Investigations
- A CT scan is the gold standard
- Arrange a chest x-ray (CXR)
- Spirometry
- Bronchoscopy
- Blood tests such as ESR (often raised), anti-nuclear factor, and rheumatoid factor (RF) may be positive
Management
- There is no cure, so treatment focuses on slowing disease progression and improving quality of life
- Pulmonary rehabilitation
- Medications like pirfenidone and nintedanib can help reduce the decline in lung function (this is done by a specialist)
- Lung transplantation may be considered for severe cases