How should I approach the management of a patient with newly diagnosed idiopathic pulmonary fibrosis?

Approach to management of a patient with newly diagnosed idiopathic pulmonary fibrosis (IPF):

• Confirm diagnosis: Ensure diagnosis is made by a multidisciplinary team with expertise in interstitial lung disease, based on clinical features, lung function tests, and high-resolution CT thorax, with pathology if indicated ¹.
• Provide information and support: Offer clear verbal and written information about the disease, investigations, prognosis, and management options, involving the patient and their family with consent ¹.
• Assess and offer pulmonary rehabilitation: At diagnosis, assess for pulmonary rehabilitation suitability, including a 6-minute walk test and quality-of-life assessment, and offer tailored rehabilitation programs with exercise and education ¹.
• Smoking cessation: Advise and support smoking cessation if the patient smokes ¹.
• Pharmacological treatment: Consider disease-modifying therapy according to lung function: for FVC >80% predicted, nintedanib is recommended; for FVC 50–80%, pirfenidone or nintedanib are options ¹. Do not use ambrisentan, azathioprine, bosentan, co-trimoxazole, mycophenolate mofetil, prednisolone, sildenafil, or warfarin to modify disease progression ¹. Oral N-acetylcysteine may be used but benefits are uncertain ¹.
• Symptom management: Manage breathlessness by assessing causes and oxygen needs; consider ambulatory or long-term oxygen therapy and pulmonary rehabilitation ¹. For cough, treat other causes and consider opioids if debilitating; thalidomide may be considered in specialist care for intractable cough ¹.
• Best supportive care: From diagnosis, provide symptom relief, manage comorbidities, withdraw ineffective or harmful therapies, and plan end-of-life care as appropriate ¹.
• Monitor disease progression: Repeat lung function tests at 6 and 12 months to assess rate of decline and prognosis; monitor more frequently if rapid deterioration is suspected ¹.
• Access to specialist nursing and palliative care: Ensure involvement of interstitial lung disease specialist nurse and access to palliative care services with multidisciplinary collaboration ¹.