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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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2.2.5 Signs and symptoms<br />

The diagnosis of IPF is rare before the age of fifty and its incidence enhances with age<br />

and the mean age at diagnosis is 67 years. Nearly 75% of the patients are males and 2/3 are<br />

smokers or previous smokers (Hyldgaard et al., 2014; Raghu et al., 2011).<br />

IPF comprises of classic signs like progressive dry cough and dyspnea, basically<br />

diminishing over months. The symptoms are present for many years in few patients before<br />

they contact a physician or are referred for investigations. Initially, the symptoms are<br />

generally experienced with regards to exercise, but later even the slightest movement can<br />

result in severe cough, dyspnea and desaturation. Though weight loss is not classical, it may<br />

be observed in the extreme stage of the disease when the respiratory work load enhances.<br />

Cancer needs to be always excluded in such cases. Few patients have experienced repetitive<br />

“airway infections” previous to the diagnosis frequently characterized by enhanced cough<br />

and phlegm, dyspnea and crackles at lung auscultation, but without exceptionally increased<br />

C-reactive protein or fever. The symptoms are rarely refined with antibiotic therapy and<br />

should possibly be depicted as minor acute worsening of IPF (Bendstrup et al., 2014).<br />

There are few subtle or non-existing clinical findings during the initiation of the<br />

disease, but may comprise of clubbing and basal velcro crackles (Cordier & Cottin, 2013).<br />

These observations may be previous to the respiratory symptoms for numerous months. It is<br />

significant to look for extra pulmonary indications of connective tissue disease, as this may<br />

exclude IPF but rather classify the lung disease as associated to the rheumatologic disease.<br />

The differential diagnosis is significant since prognosis and treatment of ILD associated to<br />

connective tissue diseases are distinct from that of IPF. The chronic respiratory deficiency<br />

usually evolves with cyanosis when pulmonary function becomes severely diminished,<br />

during the start of exercise, but may exist later also at rest. Pulmonary hypertension may lead<br />

to peripheral increased dyspnea, edemas, need of oxygen and diminishing diffusion capability,<br />

which is a comparatively common complication to severe IPF and a dangerous prognostic<br />

sign (Raghu et al., 2011).<br />

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