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SHTAC publishes new report on treatments for idiopathic pulmonary fibrosis

Published: 23 March 2015

A systematic review and economic evaluation on treatments for idiopathic pulmonary fibrosis is now available.

The report, ‘The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation’ is freely available from the NIHR Journals Library (Health Technology Assessment).

Idiopathic pulmonary fibrosis (IPF) is a serious and difficult to manage lung disease, the exact cause of which is not known. It generally affects people over 60 years of age and the main symptom is shortness of breath, which can have a considerable impact on day to day life. IPF was once thought to progress at a steady, predictable rate, but this is often not the case. Many people with IPF deteriorate rapidly, while others have periods of relative stability. In general people with the disease survive between two to five years.

The aim of treatment is to reduce symptoms and improve survival. The type of treatment offered can vary, and people with IPF can also vary in their response to the available treatments. The review included 14 studies evaluating a variety of pharmacological and non-pharmacological treatments, including azathioprine, N-acetylcysteine (NAC) (alone or in combination), pirfenidone, nintedanib, sildenafil, thalidomide, pulmonary rehabilitation, and a disease management programme. The current evidence suggests that some treatments appear to be clinically effective. The economic model results show increased patient survival for five of the pharmacological treatments, compared with best supportive care, at increased cost. General recommendations cannot be made of their cost-effectiveness owing to limitations in the evidence base. Recommendations for future research are made in the report.

For more information on SHTAC's research into respiratory diseases please visit our Research page.

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