Idiopathic pulmonary fibrosis

Respiratory (12%) Core Clinical Conditions

2B The Physician Associate is able to undertake the day to day management of the patient and condition once the diagnosis and strategic management decisions have been made by another.

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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.

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