Treatment of idiopathic pulmonary fibrosis
Antifibrotic drugs, nintedanib and pirfenidone, can slow progression of mild to moderate idiopathic pulmonary fibrosis. They are available for patients with idiopathic pulmonary fibrosis who meet specific eligibility criteria1. Choice of drug depends on patient preference, potential adverse effects and drug interactions; see the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia position statement on treatment of idiopathic pulmonary fibrosis for more information on nintedanib and pirfenidone.
Combination therapy with prednisolone, azathioprine and N-acetylcysteine is no longer recommended for treatment of idiopathic pulmonary fibrosis because it is associated with increased morbidity and mortality.
Other therapies that may be beneficial for idiopathic pulmonary fibrosis include:
- smoking cessation
- pulmonary rehabilitation
- drugs for symptom control, such as opioids and anxiolytics for dyspnoea
- long-term oxygen therapy for patients with resting hypoxaemia; however, oxygen may not improve breathlessness
- early referral for lung transplantation assessment in appropriate patients
- referral to palliative care services for symptom control, psychological care and end-of-life planning (including advance care planning).
Several comorbidities may contribute to worsening of symptoms in idiopathic pulmonary fibrosis. Comorbidities that can be treated include gastro-oesophageal reflux disease, cardiovascular disease, pulmonary hypertension and sleep-disordered breathing.