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Idiopathic Pulmonary Fibrosis (IPF) as the name suggests is a progressive disorder with no known aetiology. It is characterised by the thickening of the alveoli due to scarring resulting in cough. It is known to primarily occur in older adults over 60 years of age. The findings of IPF have a known association of Usual Interstitial Pneumonia (UIP) (Raghu et al., 2011; Kawano-Dourado & Kairalla, 2013; Wells, 2013). It has been deemed that the prognosis is generally poor when UIP has been confirmed (King et al., 2001b). The median survival rate of IPF is 50%, typically around two years after diagnosis (Raghu et al., 2011; King et al., 2001b).
Idiopathic Pulmonary Fibrosis (IPF) as the name suggests is a progressive disorder with no known aetiology. It is characterised by the thickening of the alveoli due to scarring resulting in cough. It is known to primarily occur in older adults over 60 years of age. The findings of IPF have a known association of Usual Interstitial Pneumonia (UIP) (Raghu et al., 2011; Kawano-Dourado & Kairalla, 2013; Wells, 2013). It has been deemed that the prognosis is generally poor when UIP has been confirmed (King et al., 2001b). The median survival rate of IPF is 50%, typically around two years after diagnosis (Raghu et al., 2011; King et al., 2001b).
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Source: Adopted from Myllärniemi and Kaarteenaho (2015)<br />
Though several studies have been conducted to examine the efficacy of the NAC and<br />
its anti-fibrotic effects with results found to be convincing, only one placebo-controlled trial<br />
has been conducted previously which is based on the efficacy of NAC as a monotherpy based<br />
IPF treatment (Izumi et al., 2012; Martinez et al., 2014). The initial reports on the efficacy<br />
and the use of NAC were based on the previous study by (60) which revealed that a drug<br />
regimen which combines three drugs namely prednisone, azathioprine, and NAC was found<br />
to be efficient for the treatment of IPF than a drug regimen which combines prednisone and<br />
azathioprine. However, the previous placebo controlled study by Martinez et al. (2014) did<br />
not reveal any positive effect on the endpoints of the study. On the contrary, it was revealed<br />
that IPF patients treated using NAC tends to exhibit some side effects; however, no side<br />
effects in the gastrointestinal tract were evidences. In this regard, it is evident that no studies<br />
have proved the efficacy of the treatment of IPF using NAC alone and hence a combined<br />
treatment modality has always been a topic of interest for researchers and clinicians.<br />
1.4 Problem Statement<br />
Several animal and Phase-I studies that initiated the evaluation of novel drugs in the<br />
90’s and 2000’s depicted poor efficacy especially demonstrated at the phase-III stage<br />
(Ahluwalia et al., 2014). The present decade however showed much promise when the<br />
clinical trial protocols with more defined endpoints were used for discerning effective<br />
treatment for IPF. More recently, trials have evidenced that the three drug regimen that is<br />
used for management of IPF today combining the use of prednisone, azathioprine and N-<br />
acetylcysteine is either harmful or ineffective in patients with IPF thereby not significantly<br />
contributing to the increase in survival rates of IPF (Izumi et al., 2012).Contrastingly,<br />
pirfenidone have been found to be effective in IPF patients(Azuma et al., 2005; Taniguchi et<br />
al., 2010; Noble et al., 2011; King et al., 2014b; Richeldi et al., 2014). While these studies<br />
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