Idiopathic Pulmonary Fibrosis: 5 Things to Know

There aren’t that many articles written to provide a practical guide to how a pulmonologist might approach a patient with suspected IPF – the real challenges. We found this article by Dr Aaron B. Holley; it is a gem, and certainly worth a read, especially when taking standard of care and research considerations into account when designing a clinical study. “Idiopathic Pulmonary Fibrosis: 5 Things to Know” was published by Medscape in January 2019.  Here’s our summary of the 5 key points covered:

1. Is diagnostic certainty required before prescribing antifibrotics?
Not always according to the conclusion from a post hoc analysis of the INPULSIS trial.

2. Should antifibrotics be prescribed in patients with preserved lung capacity?
The centres for advanced lung disease Dr Holley worked with seem to favour more aggressive treatments.

3. How about dual therapy with antifibrotics?
There is no verdict yet on the efficacy of using these in combination. It could be argued that concomitant use could provide better efficacy than a single agent; this will probably be studied in future clinical trials looking closely at safety as well.

4. How do we determine the best treatment for IPF-related cough?
Guidelines for treating interstitial lung disease (ILD)-related cough were released by the journal CHEST in 2018. Up to 80% of IPF patients experience chronic cough, which can have a negative effect on their quality of life. Thalidomide was considered as a therapy for IPF-related cough, and although a majority felt it was effective, the narrow therapeutic window and well-documented adverse effects proved to be limitations. Comorbid conditions and the underlying cough reflex should be the focus areas for treatment of IPF cough.

5. What are the roles of antacids and anti-reflux surgery in IPF treatment?
Until a larger study is completed, it is not clear that surgery is useful for patients with IPF who are already being treated with indicated medications. The pulmonologists that work with Dr Holley treat symptomatic GERD but do not place all patients with IPF on antacids.

Idiopathic Pulmonary Fibrosis: 5 Things to Know – Medscape – Jan 11, 2019.

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