Review articleCurrent approaches to the management of idiopathic pulmonary fibrosis

- Management of IPF aims to slow disease progression and improve quality of life.
- Two drugs have been approved for the treatment of IPF: nintedanib and pirfenidone.
- Optimal care includes symptom relief, pulmonary rehabilitation, and palliative care.
- Treatment of comorbidities should be part of the overall care of patients with IPF.
- Clinical guidelines emphasise the importance of individualised patient care.

Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal lung disease associated with dyspnoea, cough and impaired quality of life. Currently, the aims of patient care are to improve outcomes for patients by slowing the progression of the disease, extending life, and improving quality of life. A prompt, accurate diagnosis is important to enable patients to receive treatment early in the course of the disease and to be considered for lung transplantation. Two anti-fibrotic drugs, nintedanib and pirfenidone, have been shown to reduce decline in lung function in patients with IPF. In addition to pharmacological therapy, optimal management of IPF includes treatment of comorbidities, symptom relief, pulmonary rehabilitation, and palliative care. Patient education is important to enable patients to make decisions about their care and to help them manage their disease and the side-effects of anti-fibrotic drugs. Research continues into new treatments and combinations of treatments that may improve outcomes for patients with this devastating disease.