What are the recommended follow-up protocols for patients diagnosed with idiopathic pulmonary fibrosis in primary care?

In primary care, patients diagnosed with idiopathic pulmonary fibrosis (IPF) should be followed up according to the stability and progression of their disease. Recommended follow-up intervals are:

• Every 3 months or sooner if there is rapid disease progression or rapid deterioration of symptoms.
• Every 6 months or sooner if the disease is steadily progressing.
• Initially every 6 months if the disease is stable, then annually if stability continues after 1 year.

During follow-up appointments, primary care should focus on assessing lung function, oxygen therapy needs, and suitability for pulmonary rehabilitation. Smoking cessation advice should be offered to all patients who smoke. It is important to identify exacerbations and previous respiratory hospital admissions. Consideration should be given to referral for lung transplantation assessment if there are no absolute contraindications. Psychosocial needs should be evaluated, with referrals to relevant services as appropriate, including palliative care. Comorbidities such as anxiety, depression, bronchiectasis, diabetes, gastro-oesophageal reflux disease, ischaemic heart disease, lung cancer, and pulmonary hypertension should be assessed and managed according to best practice.

Discussions about lung transplantation and mechanical ventilation should ideally occur between 3 and 6 months after diagnosis, supported by an interstitial lung disease specialist nurse. Mechanical ventilation is generally not recommended for life-threatening respiratory failure in IPF.