Dr Kola Tytler MBBS CertHE MBA MRCGPClinical Lead • iatroX
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 NICE CG163.
- 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 NICE CG163.
- 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 NICE CG163.
- Smoking cessation: Advise and support smoking cessation if the patient smokes NICE CG163.
- 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 NICE CG163. Do not use ambrisentan, azathioprine, bosentan, co-trimoxazole, mycophenolate mofetil, prednisolone, sildenafil, or warfarin to modify disease progression NICE CG163. Oral N-acetylcysteine may be used but benefits are uncertain NICE CG163.
- Symptom management: Manage breathlessness by assessing causes and oxygen needs; consider ambulatory or long-term oxygen therapy and pulmonary rehabilitation NICE CG163. For cough, treat other causes and consider opioids if debilitating; thalidomide may be considered in specialist care for intractable cough NICE CG163.
- Best supportive care: From diagnosis, provide symptom relief, manage comorbidities, withdraw ineffective or harmful therapies, and plan end-of-life care as appropriate NICE CG163.
- 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 NICE CG163.
- Access to specialist nursing and palliative care: Ensure involvement of interstitial lung disease specialist nurse and access to palliative care services with multidisciplinary collaboration NICE CG163.