Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias, and poses significant clinical challenges. IPF diagnosis is based on clear-cut computed tomography (CT) and histopathological criteria, in an appropriate clinical context. The diagnostic criteria include: 1) exclusion of known causes of interstitial lung disease (including connective tissue disease); 2) usual interstitial pneumonia pattern on high-resolution CT in patients not subjected to surgical lung biopsy; and 3) specific combinations of high-resolution CT with pathological patterns in case of surgical lung biopsy. Improved diagnosis of IPF may help physicians to reduce the delay before an accurate diagnosis is made and increase patient awareness and access to adequate information, follow-up and treatment.KEYWORDS: Diagnosis, idiopathic pulmonary fibrosis I diopathic interstitial pneumonias (IIPs) are a group of diseases also known as interstitial lung diseases (ILDs) in the absence of any known cause and of clinical manifestations other than limited to the lung [1]. Idiopathic pulmonary fibrosis (IPF) is the most common of the IIPs, accounting for 50-60% of diagnosed cases [2] and represents both the most frequent and most severe of all ILDs. Epidemiological studies suggest that IPF is more common in males with onset usually in middle or older age (prevalence peaks at age 65-79 yrs) [2], but it has no distinct geographic distribution and does not distinguish between particular races or ethnic groups [3]. It is usually sporadic but the notion of prior familial cases can be found in 3-10% of cases. The main known risk factors that are associated with disease development include smoking (either current or past), some environmental factors, and genetic predisposition [3].IPF is characterised by a distorted alveolarcapillary barrier architecture (several elements are involved including epithelial and endothelial cell apoptosis [4], infiltration of inflammatory cells into interstitial and alveolar spaces, fibroblast proliferation and excessive deposits of interstitial collagen) leading to an impaired gas exchange [5,6]. The specific molecular and cellular mechanisms, cause of disease onset and disease progression are still unknown. Despite some limits, animal models of pulmonary fibrosis can be of invaluable help for evidencing some of the pathogenic processes at play in IPF [7]. IPF is now considered as a distinct entity with lesions that vary in age and location. The established view that IPF was a disease in which fibrosis was directly caused by chronic inflammation has been challenged by two main arguments: 1) clinical measurements of inflammation failed to correlate with stage or outcome, and 2) potent anti-inflammatory therapy does not improve outcome [8].The disease course in IPF is variable. Some patients may remain stable for long periods of time but a significant proportion demonstrate slow progression and others experience acute exacerbations leading to respiratory failure and death. In some pat...