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):

• Initial assessment and diagnosis: Confirm diagnosis with a multidisciplinary team including a respiratory physician, radiologist, specialist nurse, and others with interstitial lung disease expertise, using clinical features, lung function tests, chest X-ray, and high-resolution CT thorax. Consider biopsy if diagnosis is uncertain ¹.
• Information and support: Provide clear verbal and written information about the disease, investigations, prognosis, and management options. Involve the patient and their carers with consent ¹.
• Smoking cessation: Offer advice and support to stop smoking if the patient smokes ¹.
• Monitor disease progression: Measure lung function (spirometry and gas transfer) at diagnosis, 6 months, and 12 months to assess rate of decline and prognosis. Repeat more frequently if rapid deterioration is suspected ¹.
• Pulmonary rehabilitation: Assess for pulmonary rehabilitation at diagnosis and at 6- or 12-month intervals. Offer tailored exercise and education programs accessible to the patient 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.
• Oxygen therapy: Assess for ambulatory and long-term oxygen therapy if breathless on exertion or at rest, including after hospitalisation ¹.
• Cough management: Treat other causes of cough such as reflux or post-nasal drip. Consider opioids for debilitating cough. Discuss thalidomide treatment with a specialist if cough is intractable, noting it is off-label ¹.
• Disease-modifying pharmacological treatment: Offer nintedanib for patients with forced vital capacity (FVC) above 80% predicted. For FVC 50-80%, consider pirfenidone or nintedanib as per NICE technology appraisals. Do not use ambrisentan, azathioprine, bosentan, co-trimoxazole, mycophenolate mofetil, prednisolone, sildenafil, or warfarin to modify disease progression ¹.
• Access to palliative care: Ensure collaboration between healthcare professionals and palliative care teams to support the patient and family ¹.