Idiopathic pulmonary fibrosis in adults: diagnosis and management

Evidence

We found 24 studies in a search for randomised controlled trials and systematic reviews published between 13 February 2015 and 22 September 2016. We also considered 2 additional studies identified by members of the guideline committee who originally worked on this guideline.

We also considered evidence identified in a previous surveillance 12 years after publication of the guideline.

From all sources, we considered 38 studies to be relevant to the guideline.

We also checked for relevant ongoing research, which will be evaluated again at the next surveillance review of the guideline.

See appendix A: summary of evidence from surveillance and references for all evidence considered.

Views of topic experts

We considered the views of topic experts, including those who helped to develop the guideline.

Views of stakeholders

Stakeholders commented on the decision not to update the guideline. Overall, 7 stakeholders commented. See appendix B for stakeholders' comments and our responses.

One agreed and 6 disagreed with this proposal. Of the 6 who disagreed, 1 provided comments that were unclear as to why they did not agree with the proposal not to update. Three stakeholders who disagreed with the decision to not update the guideline suggested updating the guideline in accordance with NICE technology appraisal guidance on nintedanib, and asked for clearer statement around the lack of benefit of N‑acetylcysteine.

We responded that we will amend the guideline to add a cross-reference to NICE technology appraisal guidance TA379 published in January 2016 from recommendations in section 1.5 in NICE guideline CG163. Concerning N‑acetylcysteine, NICE guideline CG163 recommends 'advise the person that oral N‑acetylcysteine is used for managing idiopathic pulmonary fibrosis, but its benefits are uncertain' (recommendation 1.5.13). Because the recommendation already acknowledges uncertainty about the benefits of this drug, and no new safety concerns have been raised in review of evidence about its use, there is no impact on current recommendations at this time. This area will be examined again at the next surveillance review of the guideline.

Two stakeholders who also disagreed with the decision to not update the guideline commented that patients' and carers' values and preferences need to be established. In the current surveillance review, none of the new evidence considered was thought to have an impact on the current recommendations on patients' information and support.

NICE has produced guidance on the components of good patient experience in adult NHS services. The related recommendations are in the NICE guideline on patient experience in adult NHS services.

Three stakeholders disagreed with the decision to remove 3 of the research recommendations from the NICE version of the guideline and NICE research database because of relevant ongoing trials; therefore these recommendations will be retained.

See ensuring that published guidelines are current and accurate in developing NICE guidelines: the manual for more details on our consultation processes.

NICE Surveillance programme project team

Sarah Willett
Associate Director

Philip Alderson
Consultant Clinical Adviser

Katrina Sparrow
Technical Adviser

Maryam Gholitabar
Technical Analyst

The NICE project team would like to thank the topic experts who participated in the surveillance process.

ISBN: 978-1-4731-2468-4