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patient in the intervention group and three in the control group. This might relate the
likeliness of the combined treatment which reduces mortality rate to some extent in the
intervention group. In all these studies, there is no evidences of reducing the rate of mortality;
however, with the reduction of adverse effects of IPF all studies warrant that the combined
treatment, to some extent aid reduction of rate of mortality in patients with IPF.
5.3 Management framework for IPF
There have been significant advances in the management of IPF clinically since the
development of the evidence-based guidelines in 2011 92 . Several weak and conditional
recommendations have been received with respect to the treatment of IPF wherein the
guidelines developed in the year 2011 has been reviewed with recommendations provided 93 .
However, there are no interventions till date which could act as recommendations for the
treatment. Till date several recommendations have been made with respect to the treatment of
IPF with novel agents such as nintedanib and pirfenidone. However, there are chances for
future researches to open the venues for the developing Pirfenidone and N-acetylcysteine as a
combined drug treatment for IPF. However, these researches are still in its infancy and hence
medical practitioners should look into other management of IPF appropriately.
The results of the study by Behr et al 94 revealed that though there are safety
constraints with respect to the use of N-acetylcysteine with pirfenidone in patiens with IPF,
the findings of the research should be considered with caution as the sample size is relatively
less. Furthermore, this is applicable for all the researches considered for the systematic
review as the sample sizes of all these studies are relatively less in number. However, to
reveal a more clear evidence on the examination of efficacy of N-acetylcysteine and
pirfenidone a four armed study as specified by Raghu 95 needs to be performed which should
comprise of the following- control group; pirfenidone monotherapy; NAC monotherapy; and
combination therapy. In addition, the present research suggests the use of high sample size so
92
https://www.thoracic.org/statements/resources/interstitial-lung-disease/IPF-Full-length.pdf
93
https://www.thoracic.org/statements/resources/interstitial-lung-disease/IPF-Full-length.pdf
94
https://www.ncbi.nlm.nih.gov/pubmed/27161257
95 http://www.thelancet.com/pdfs/journals/lanres/PIIS2213-2600(16)30327-7.pdf
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