New antifibrotic drugs have revolutionised the management of idiopathic pulmonary fibrosis but it remains a progressive and fatal disease that requires people to prepare for the worst, according to a new position statement from the Thoracic Society of Australia & New Zealand and the Lung Foundation.
Published in the MJA, the position statement on IPF says antifibrotic therapies such as nintedanib and pirfenidone should be considered as first line therapy for patients with mild to moderate IPF, but not for severe or very mild asymptomatic disease.
Non-drug approaches such as pulmonary rehabilitation, oxygen therapy and lung transplantation are just as important and should be used early in what is still an incurable and fatal disease, the statement recommends.
Attention is also needed to management of patients’ co-morbidities such as reflux disease and pulmonary hypertension, although there is little evidence to support the use of therapies such as antacids and vasodilators respectively, it notes.
Developed by a panel of experts who conducted a literature review, the position statement is not intended to be a set of guidelines, according to the authors led by Dr Helen Jo, a Respiratory Physician of the Royal Prince Alfred Hospital in Sydney.