Which investigations are essential for confirming a diagnosis of idiopathic pulmonary fibrosis?

Essential investigations for confirming a diagnosis of idiopathic pulmonary fibrosis (IPF) include:

• Detailed clinical history and examination to identify typical features such as age over 45 years, persistent breathlessness on exertion, persistent cough, bilateral inspiratory crackles, and clubbing of the fingers.
• Blood tests to exclude alternative diagnoses including lung diseases related to environmental/occupational exposure, connective tissue diseases, and drug-induced lung disease.
• Lung function testing including spirometry and gas transfer measurement to assess for restrictive or sometimes obstructive patterns.
• Chest X-ray as an initial imaging modality to review lung abnormalities.
• High-resolution computed tomography (HRCT) of the thorax, which is critical for identifying the characteristic radiological patterns of IPF.
• Multidisciplinary team (MDT) consensus diagnosis based on clinical, lung function, and radiological findings.
• If the MDT cannot make a confident diagnosis from the above, further investigations such as bronchoalveolar lavage, transbronchial biopsy, and/or surgical lung biopsy may be considered, weighing the risks and benefits with the patient.

Diagnosis should only be confirmed with MDT agreement involving respiratory physicians, radiologists, histopathologists, specialist nurses, and coordinators with expertise in interstitial lung disease.

These investigations and diagnostic steps are essential to differentiate IPF from other interstitial lung diseases and to ensure accurate diagnosis and management.

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