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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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histological pattern of UIP in the examination of lung tissue acquired by surgical lung biopsy,<br />

or radiological confirmation of a UIP pattern on the high resolution computed tomography<br />

(HRCT) or both.<br />

The pulmonologists, pathologists and radiologists accomplished that the<br />

multidisciplinary assessment in the diagnosis and management of DILD can enhance the<br />

diagnostic precision, which currently is a broadly assumed proposal for demonstrating the<br />

diagnosis (Flaherty et al., 2007; Raghu et al., 2011).<br />

2.2.8.1 Clinical Characteristics and Additional Tests<br />

The clinical representation of IPF is generally indicated by progressive dyspnea on<br />

exertion and has a subtle onset, usually assisted by non-productive cough. The occurrence of<br />

the symptoms is gradual, but aggravates over time. There is an inconsistent retard between<br />

the onset of symptoms and the final diagnosis, and it may occur between 6 months and two<br />

years (Kim, 2006). An auxiliary diagnosis is traced to be caused by the existence of systemic<br />

symptoms/signs. In 90% of patients crackles can be heard on auscultation and in 50% of<br />

patients acropachy is determined (Xaubet et al., 2003a).<br />

No particular laboratory deformities exist for this disease. Nevertheless, certain signs<br />

or symptoms of connective tissue diseases in their deprivation, serological autoimmune tests<br />

should be conducted in all patients.2 The positive rheumatoid factor or anti-nuclear<br />

antibodies can be discerned in up to 20% of IPF cases (Xaubet et al., 2003a). The existence<br />

of serum specific IgG should be evaluated consistently against the antigens that can most<br />

frequently cause hypersensitivity pneumonitis, since its clinical illustrations are at times<br />

similar to those of IPF. If any of these are positive, in the situation of reasonable exposure<br />

and bronchoalveolar lavage (BAL) with an enhanced lymphocyte count, an irritation test<br />

against the antigen in question and/or surgical lung biopsy should be conducted, in order to<br />

affirm or reject the diagnosis of chronic hypersensitivity pneumonia (Xaubet et al., 2003a).<br />

The probability of employing new biomarkers in the depiction and diagnosis of this disease<br />

has also obtained concern in the current years. Few biomarkers like KL-6, SP-A and SP-D,<br />

circulating fibrocytes and metalloproteinases 1 and 7 are being presently researched (Rosas et<br />

al., 2008; van den Blink et al., 2010).<br />

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