What are the indications for referring a patient with suspected idiopathic pulmonary fibrosis to a specialist?

Indications for referring a patient with suspected idiopathic pulmonary fibrosis (IPF) to a specialist include:

• Presence of clinical features suggestive of IPF such as age over 45 years, persistent breathlessness on exertion, persistent cough, bilateral inspiratory crackles on chest auscultation, and clubbing of the fingers.
• Abnormal lung function tests, typically showing a restrictive pattern or impaired gas transfer.
• Abnormal chest X-ray findings suggestive of interstitial lung disease.
• Need for further diagnostic evaluation including high-resolution CT of the thorax to confirm suspicion.
• Uncertainty in diagnosis after initial clinical, radiological, and lung function assessment, requiring multidisciplinary team evaluation.
• Consideration of bronchoalveolar lavage, transbronchial biopsy, or surgical lung biopsy if diagnosis remains unclear after initial assessments.
• Referral to a multidisciplinary team with expertise in interstitial lung disease for diagnosis confirmation and management planning.

Referral should be made promptly once IPF is suspected based on these features to enable timely diagnosis and management by specialists experienced in interstitial lung disease.

Summary: Refer patients with suspected IPF to a respiratory specialist when clinical features, lung function tests, or imaging suggest IPF, or when diagnosis is uncertain and requires specialist multidisciplinary assessment and possible invasive diagnostic procedures.

NICE CG163