Treatment for Idiopathic Pulmonary Fibrosis

As everyone working in this field knows, the prognosis of idiopathic pulmonary fibrosis (IPF) is quite dismal (3 years survival of 50%). There have been many treatment regimens that have been suggested, but it is not clear whether any of them are of any use.

The latest issue of the New England Journal of Medicine (NEJM) has 3 articles and one editorial on this subject. The last one on acetyl-cysteine confirms that it does not really work.

However one more phase III trial by Talmadge King’s group on the use of pirfenidone, conducted because the US FDA was not convinced about its efficacy given the conflicting results from previous trials, shows that it does improve decline in lung function and overall survival.

The paper that however does bring a smile and some more hope is the first one by Richeldi Luca et al that shows the usefulness of nintedanib (where do come up with these names) in improving the reduction in FVC in patients with IPF.

There is a lovely short commentary / editorial that puts all these papers in perspective and even addresses the question of whether both nintedanib and pirfenidone should be used together and whether that would help even more or not. This is an interesting thought.

All of these are must reads for all those who have patients with IPF whose care they are responsible for.


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