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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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Figure 5: Possible Mechanisms of Familial IPF<br />
Source: Adopted from Gunther et al. (2012)<br />
2.2.7 Clinical Course of IPF<br />
2.2.7.1 Subclinical IPF<br />
The symptoms forego diagnosis that is well observed by a median of 1 to 2 years<br />
(Nagai et al., 1999; Nicholson et al., 2000; King et al., 2001a; Collard et al., 2003; Jegal et<br />
al., 2005; Bjoraker et al., 1998; Nagai et al., 1998), and radiographic confirmation of the<br />
disease may even forego the symptoms, indicating “subclinical” intervals of disease that are<br />
not well described (Nagai et al., 1998). The development of asymptomatic to symptomatic<br />
IPF may happen over years to decades (El-Chemaly et al., 2011). Early lung fibrosis is<br />
asymptomatic and has been progressively identified and described in family members of the<br />
affected individuals with familial pulmonary fibrosis, basically in those with a history of<br />
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