The goal of this review is: to help recognize the typical pattern of usual interstitial pneumonia (UIP), which is predominantly seen in idiopathic primary fibrosis (IPF) to describe the role of High-Resolution Computed Tomography in establishing the final diagnosis of IPF

IPF is the most common entity of idiopathic interstitial pneumonias. It is defined as achronic progressive fibrotising lung disease of unknown cause and it occurs in older patients. With the median survival from 2 to 3 years it has apoorer prognosis than non-specific interstitial pneumonia (NSIP) and other interstitial pneumonias. Although the primary cause of the condition stays unknown, the current theory suggests that IPF is caused by tiny foci of acute lung injury which occur and reoccur in the interstitium over many years. Inflammation...

Following the official 2011 ATS/ERS/JRS/ALAT diagnostic criteria for IPF, the characteristic HRCT UIP pattern includes reticular opacities, often associated with traction bronchiectasis (Fig. 1) in bibasal and peripheral distribution (Fig. 3) and the appearance of honeycombing (Table 1), which is critical for making adefinite diagnosis. Honeycombing (Fig. 2) appears as variable-sized cystic spaces, with irregularly thickened walls composed of fibrous tissue. Several studies have shown, that honeycombing is consistent with the histopathological diagnosis of UIP. Furthermore, honeycombing has also apredictive value, since it portends ashorter...

For the final diagnosis of IPF other known causes of interstitial lung disease have to be ruled out. The typical UIP pattern on HRCT is sufficient for setting the final diagnosis of IPF without the need of surgical lung biopsy. The extent of disease, presence of honeycombing and traction bronchiectasis are predictive for survival and mortality of IPF patients.

Vetešková Štefánia, M.D. Department of Radiology, Jessenius Faculty of Medicine in Martin, Comenius University inBratislava, Kollarova 2, 036 59 Martin, Slovakia Zeleňák Kamil, M.D., PhD., FCIRSE, Associate Professor Head of the department of Radiology, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Kollarova 2, 036 59 Martin, Slovakia Števík Martin, M.D., PhD. Department of Radiology, Jessenius Faculty of Medicine in Martin, Comenius University inBratislava, Kollarova 2, 036 59 Martin, Slovakia Vorčák Martin, M.D. Department of Radiology, Jessenius Faculty of Medicine in Martin, Comenius...

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