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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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IIPs (specifically UIP) in the older age groups<br />
Gender<br />
CVDs, Lofgren's syndrome in women<br />
Tobacco smoking DIP, respiratory bronchiolitis related IIP<br />
Occupational exposure Hypersensitivity pneumonitis, pneumoconiosis<br />
Onset of symptoms Acute, subacute or chronic<br />
<strong>Drug</strong> intake<br />
cytotoxics, amiodarone, NFT<br />
Family history sarcoidosis, UIP<br />
Febrile illness infections, SLE, vasculitis, sarcoidosis, TPE<br />
Hemoptysis vasculitis (WG), pulmonary haemorrhage, hemosiderosis,<br />
Goodpasture's<br />
Athralgia/arthritis sarcoidosis (ankle arthritis), RA(hands)<br />
Skin lesions<br />
Sarcoid lesions, EN, vasculitic ulcer, subungual infarct, heliotrope,<br />
PSS, rheumatoid nodule, butterfly rash<br />
Eye involvement Uveitis, conjunctivitis, scleritis, xerophthalmia in sarcoidosis,<br />
CVD, WG, Sjogren's<br />
Peripheral<br />
TB, sarcoid, fungal infection, HIV<br />
lymphadenopathy<br />
Digital clubbing UIP<br />
Raynaud's<br />
PSS<br />
phenomenon<br />
Source: Source: Adopted from Pande (2013)<br />
In view of the prognostic distinctions that prevail amongst the IIPs, precise diagnosis<br />
of IPF is crucial. Clinical diagnosis of IIPs presently is established on a comprehensive<br />
medical history along with a physical examination. Prior identification of IPF certainly<br />
usually begins with a high degree of clinical intuition and good clinical awareness<br />
accompanied by chest radiographs and lung function tests. In IIP presumed patients HRCT is<br />
conducted. HRCT is now an essential component of the diagnostic technique for IIPs (fig. 1),<br />
as explored below in detail, wherein a constant technique in which pulmonologists,<br />
radiologists, pathologists and thoracic surgeons are all involved in a significant role. The<br />
requirement for SLB in as many as 50% of patients with IIP may be abolished though by the<br />
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