About idiopathic pulmonary fibrosis

Idiopathic Pulmonary Fibrosis (IPF) is a progressive and often fatal condition. In a 2016 publication it was estimated that over 5,000 new IPF cases were diagnosed each year in the UK and over 30,000 people were living with the disease.⁶⁴

Patients with this condition often have disabling symptoms of breathlessness and cough.

Anti-fibrotic (AF) therapy

• Anti-fibrotic (AF) therapy has been shown to reduce loss of lung function and preserve life when used effectively in IPF.^66,67,70
• These medicines (pirfenidone and nintedanib) have a high side effect profile, however, and do not improve the symptoms of IPF.^66,67
• Nintedanib has also shown benefit for patients with other Progressive Fibrosing Interstitial Lung Diseases.⁶⁵ 
• Patient awareness of these issues and the risks and benefits of taking treatment is important to ensure appropriate adherence with therapy.
• Anti-fibrotic therapy should only be prescribed in secondary care.
• For GP practices these medicines should be added to the patient record to highlight prescribing in secondary care (as an ‘outside issue’). This allows interactions to be checked when prescribing for another condition.

Good patient care of people with IPF will require effective communication between secondary and primary care clinicians alongside the use of respiratory support services such as Pulmonary Rehabilitation and secondary care Respiratory Nurse Specialists where available.

End of life care can often be managed in primary care. GP practice teams should ensure that anticipatory care plans and medicines are in place when approaching end of life, to allow timely access to these when required.