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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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tolerability and safety of pirfenidone and acetylcysteine combination therapy in IPF. The<br />

research considered several researches wherein the research by Sakamoto et al 73 was also<br />

taken into account by Behr et al 74 . It is revealed that in European nations, according to 75 and<br />

76<br />

, more than one third of patients were reported to have received both acetylcysteine and<br />

pirfenidone. After the PANTHER study 77 , there is a decline in the use of acetylcysteine;<br />

however, only two researches by Sakamoto et al 78 and Oltmanns et al 79 have examined the<br />

combined efficacy of pirfenidone and acetylcysteine. In this context, Behr et al 80 conducted a<br />

PANORAMA study to investigate the tolerability and safety of oral acetylcysteine with<br />

pirfenidone for the treatment of IPF.<br />

The examination of all the studies revealed contrasting information regarding the efficacy of<br />

the combined drug therapy. Though it is revealed that several clinical trials have been<br />

conducted for treating IPF, the disease still remains to be a fatal and a progressive disease 81 82 .<br />

However, with the aim to provide a novel combination that could effectively treat IPF, the<br />

study by Sakamoto et al 83 examined the combination therapy of pirfenidone and inhaled NAC<br />

which revealed that the treatment improved the values of FVC in more than 45 per cent of<br />

patients with advanced IPF. It is further revealed that in the intervention group receiving<br />

combined treatment, the value of PFS was better than those in the control group receiving<br />

Pirfenidone alone. The results of the research further reveal the fact that in patients with<br />

advanced IPF, inhaled NAC and pirfenidone decreased the risks of poor outcomes. However,<br />

73<br />

https://www.ncbi.nlm.nih.gov/pubmed/25639750<br />

74 https://www.ncbi.nlm.nih.gov/pubmed/27161257<br />

75 Behr J, Kreuter M, Hoeper MM, et al. Management of patients with idiopathic pulmonary fi brosis in clinical<br />

practice: the INSIGHTS-IPF registry. Eur Respir J 2015; 46: 186–96<br />

76<br />

Oltmanns U, Kahn N, Palmowski K, et al. Pirfenidone in idiopathic pulmonary fi brosis: real-life experience<br />

from a German tertiary referral center for interstitial lung diseases. Respiration 2014; 88: 199–207<br />

77<br />

Martinez FJ, de Andrade JA, Anstrom KJ, King Jr TE, Raghu G, for the Idiopathic Pulmonary Fibrosis<br />

Clinical Research Network. Randomized trial of acetylcysteine in idiopathic pulmonary fi brosis. N Engl J Med<br />

2014; 370: 2093–101.<br />

78<br />

https://www.ncbi.nlm.nih.gov/pubmed/25639750<br />

79<br />

Oltmanns U, Kahn N, Palmowski K, et al. Pirfenidone in idiopathic pulmonary fi brosis: real-life experience<br />

from a German tertiary referral center for interstitial lung diseases. Respiration 2014; 88: 199–207<br />

80<br />

https://www.ncbi.nlm.nih.gov/pubmed/27161257<br />

81 King TE Jr, Albera C, Bradford WZ, Costabel U, Hormel P, Lancaster L, Noble PW, Sahn SA, Szwarcberg J,<br />

Thomeer M et al. INSPIRE Study Group. Effect of interferon gamma-1b on survival in patients with idiopathic<br />

pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet 2009; 374: 222–8.<br />

82<br />

King TE Jr, Behr J, Brown KK, du Bois RM, Lancaster L, de Andrade JA, Stähler G, Leconte I, Roux S,<br />

Raghu G. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am.<br />

J.Respir.Crit.Care Med. 2008; 177: 75–81.<br />

83 https://www.ncbi.nlm.nih.gov/pubmed/25639750<br />

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