Diagnosis

• Hx suggestive:
  • Environmental, occupational and drugs exposure (methotrexate, amiodarone, ergot)
  • >45
  • Persistent SOB
  • Bilateral inspiratory crackles
  • Clubbing of fingers
  • Normal or impaired spirometry, usually restrictive but can be obstructive
  • Only diagnose with MDT input and investigation below

Investigation

• Bloods tests to exclude: connective tissue disease
• X-ray and CT
• Lung function tests (Spirometry and TLCO)
• If still unable to diagnose: Bronchioalveloar lavage / transbronchial or surgical biopsy

Management

Conservative

• Smoking cessation
• Assess for pulmonary rehabilitation: 6 min walk test with pulse oximetry + QoL assessment
• “Best Supportive Care” including:
  • LTOT / Ambulatory oxygen
  • Pulmonary rehabilitation

Pharmacological

• Symptomatic relief: benzodiazepines /opioids
• No evidence for disease modify pharmacological intervention

Surgical:

• Lung transplantation if no contra-indication
• Palliative care

NICE Source: CG163 Idiopathic pulmonary fibrosis in adults: diagnosis and management. Summary compiled by Dr D P Sheppard MBBS.