Idiopathic pulmonary fibrosis

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Definition Aetiology Pathophysiology Risk factor Sign and Symptoms Investigations Management

Idiopathic Pulmonary Fibrosis (IPF) is a chronic and progressive lung disease characterised by the scarring (fibrosis) of the lung tissue. The term "idiopathic" means that the cause of the disease is unknown, making IPF a diagnosis of exclusion. It primarily affects the interstitial spaces of the lungs, impairing their ability to expand and contract efficiently.

• Unknown causes of IPF.

• Could be the following:

• Genetic susceptibility.

• Environmental exposures (such as cigarette smoke and occupational hazards).

• Viral infections.

• Abnormal immune response.

There is an overproduction of collagen (an extracellular matrix protein) in the lung tissue. This process impairs the lung's gas exchange ability which overtime causes a decline in lung function.

• Age > 50 years.

• Gender: men are more at risk.

• Smoker.

• Work related e.g. such as asbestos or wood dust.

• Family history of pulmonary fibrosis.

• Persistent non productive cough.

• SOBOE.

• Tiredness.

• Weight loss.

• Chest discomfort or chest pain.

• CT scan is the gold standard.

• CXR.

• Spirometry.

• Bronchoscopy.

• blood tests: ESR will usually be raised, anti-nuclear factor might be positive, rheumatoid factor (RF) might also be positive.

• Currently no cure, the main goal is to slow down disease progression, alleviate symptoms, and improve quality of life.

• Pulmonary rehabilitation.

• Medications: e.g. pirfenidone and nintedanib, have shown efficacy in reducing the decline in lung function.

• Lung transplantation may be considered in severe cases.