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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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al., 1979; Keogh & Crystal, 1980). Patients have an enhanced risk of death if the Vࡆo2max<br />

less than 8.3 ml/kg/min at baseline (Fell et al., 2010). The 6MWT has become the most<br />

extensively employed exercise test in most of the lung diseases, including IPF, given its easy<br />

execution and reproducibility (Eaton et al., 2005; Kadikar et al., 1997). Distance walked<br />

(Lederer et al., 2006b; Caminati et al., 2009) and desaturation (Hallstrand et al., 2005;<br />

Flaherty et al., 2006) during the 6MWT both have been observed to anticipate mortality, and<br />

in one study a composite of the product of distance and desaturation anticipated mortality<br />

better than either of the measures alone (Lettieri et al., 2006b). Abnormal heart rate recovery<br />

after 1 minute of rest after the 6MWT also may be a unique and strong indicator of mortality<br />

(Lettieri et al., 2006b). Current data exhibits that a significant modification in 6MWD is<br />

extremely predictive of mortality (i.e., a decrease in 6MWD > 50 m over 24 wk is related<br />

with a fourfold enhancement in the risk of death at 1 year [P < 0.001]) (Bois et al., 2011). It<br />

has been recommended additionally that the least significant distinction for 6MWD is nearly<br />

30 m (i.e, patients can distinguish the smallest modification in distance as distinct from the<br />

earlier test and that would authorize, an alteration in the management in the lacking of<br />

annoying side effects and lavish costs) (Swigris et al., 2010; Bois et al., 2011). The<br />

confirmation to the effectiveness of desaturation during exercise testing for anticipating<br />

mortality can be done by two other exercise tests, the 15-step and 4-minute step tests (Shitrit<br />

et al., 2009; Stephan et al., 2007).<br />

Pathologic Predictors:<br />

The histopathologic pattern is UIP that recognizes IPF, and it conveys the worst<br />

prognosis amongst the idiopathic interstitial pneumonias (American Thoracic Society &<br />

European Respiratory Society, 2002; Bjoraker et al., 1998; Nicholson et al., 2000). Biopsies<br />

from distinct lobar specimens in the same patient fascinatingly may exhibit histologic<br />

discordance, that is, nonspecific interstitial pneumonia in one area and patterns of UIP in<br />

another area (i.e., discordant UIP). Discordant UIP category patients exhibit survival, clinical<br />

and physiologic characteristics comparable to those observed in the concordant UIP category,<br />

and significantly, the prognosis in both concordant and discordant UIP categories was<br />

notably worse than that of the concordant nonspecific interstitial pneumonia category<br />

(Flaherty et al., 2001; Monaghan et al., 2004).<br />

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