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 exposures, 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 pattern of usual interstitial pneumonia (UIP) associated with 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 to obtain histopathological confirmation.

Diagnosis should only be confirmed with MDT consensus involving respiratory physicians, radiologists, histopathologists, and specialist nurses with expertise in interstitial lung disease to ensure accuracy and appropriate interpretation of findings.

These steps are essential to exclude other causes and confirm IPF diagnosis accurately.

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