There are various principles to be mindful of when prescribing nintedanib or pirfenidone for patients with IPF. These include the following:

  • Nintedanib and pirfenidone should only be prescribed by a clinician with experience of treating IPF and they should monitor the benefit of AF medicines
  • Nintedanib and pirfenidone are approved for restricted use in Scotland for patients with a predicted forced vital capacity (FVC) less than or equal to 80% , 
  • The two available drugs have similar efficacy and have different side effect profiles - most patients who cannot tolerate one therapy will tolerate the other
  • Ensure appropriate treatment choice including drug interactions and potential side effect profile
    • Nintedanib is associated with liver injury and requires blood monitoring monthly for the first three months then six monthly thereafter  - common side effects include diarrhoea, nausea, abdominal pain, weight loss and decreased appetite
    • Pirfenidone is associated with hepatic injury and requires blood monitoring monthly for the first six months then three monthly thereafter  - common side effects include nausea, indigestion, photosensitivity and rash
  • Primary care clinicians should refer the patient presenting with these side effects to the consultant.
  • Shared care agreements are variable across Scotland, so it is important to establish who is responsible for blood monitoring