Usual interstitial pneumonia

Last revised by Frank Gaillard on 27 Nov 2023

Usual interstitial pneumonia (UIP) is a histopathologic and radiologic pattern of interstitial lung disease, which is the hallmark pattern for idiopathic pulmonary fibrosis (IPF)

On imaging, usual interstitial pneumonia usually presents with a lung volume loss and an apicobasal gradient of peripheral septal thickening, bronchiectasis, and honeycombing. 

This article will focus solely on the usual interstitial pneumonia pattern as a radiological or histopathological descriptor, for further discussion in the clinical aspects, please refer to the parental article on the specific underlying clinical diagnosis (e.g. idiopathic pulmonary fibrosis).    

In the past, the term usual interstitial pneumonia was used synonymously with idiopathic pulmonary fibrosis. However more recently the term idiopathic pulmonary fibrosis has been applied solely to the clinical syndrome associated with the morphologic pattern of UIP, with the specific exclusion of entities such as non-specific interstitial pneumonia (NSIP) and desquamative interstitial pneumonia (DIP) 1

The histological diagnosis of UIP is based on temporal and spatial heterogeneity, which is the identification of fibrotic lesions at different stages (fibroblastic infiltrates, mature fibrosis, and honeycombing) within the same biopsy specimen and architectural distortion. Honeycombing, particularly if it involves more than 5% of the lung volume, is an almost 100% specific finding. On a typical biopsy, there are areas of normal lung alternating with interstitial fibrosis and honeycombing. The distribution of UIP characteristically is with an apicobasal gradient with basal and peripheral (subpleural) predominance, although it is often patchy.

Inflammation is absent or mild and mostly limited to the areas of honeycombing 1-12.

UIP pattern of interstitial lung disease can be seen in idiopathic pulmonary fibrosis or secondary to underlying systemic diseases. These would include:

In practice, the diagnosis is usually made in a multidisciplinary approach involving chest physicians, radiologists and pathologists with expertise in interstitial lung disease 12.

Plain film features are non-specific. While chest radiographs can be even normal in patients with very early disease, in advanced disease, it may show decreased lung volumes and basal fine to coarse reticulation. Usually, due to the more extensive involvement of the lower lobes, the major fissure is shifted inferiorly which is best seen on the lateral chest radiograph.

When describing imaging features, the term UIP pattern is often used, which has specific diagnostic criteria on HRCT 16. The positive predictive value of CT in the diagnosis of UIP is high and ranges from 70-100% 1. Similar to the pathology specimen, cross-sectional imaging also reveals heterogeneity, with patchy areas of fibrosis alternating with areas of normal lung 5.

Typical features include 1,5:

  • honeycombing: particularly if it involves more than 5% of the lung parenchyma, is highly specific for UIP. In general, UIP can be divided into two groups, those with <5% honeycombing and those with >5% honeycombing. It mainly reflects the stage and severity of the disease. Those with less than 5% honeycombing may pose diagnostic difficulty as differentiation from NSIP on imaging can be impossible; however, these still follow similar prognosis as other UIP patients 2

  • reticular opacities: in the immediate subpleural lung, often associated with honeycombing and traction bronchiectasis, with peripheral and lower lobe predominance, is considered a very good differentiating feature from patients with NSIP and concurrent emphysema 2

  • reticular opacity-to-ground glass opacity ratio: one or greater

    • ground-glass opacities: usually less extensive than the reticular pattern and almost never seen in isolation - usually happens in areas of reticulation or honeycombing

  • lung architectural distortion: which reflects lung fibrosis and is often prominent

  • lobar volume loss (predominantly lower lobes) is seen in cases of more advanced fibrosis

In recent times some authors have suggested certain signs within a UIP pattern more suggestive of it being due to connective tissue disorder interstitial lung disease over idiopathic pulmonary fibrosis 22

These include

  • straight-edge sign: fibrosis isolated to the lung bases with a sharp demarcation best seen on coronal images in the craniocaudal plane between fibrotic lung inferiorly and spared lung superiorloy, without significant extension along the lateral margins of the lungs

  • anterior upper lobe sign: fibrosis concentrated along the anterior aspect of the upper lobes with concomitant lower lobe involvement but relative sparing of the other aspects of the upper lobes

  • exuberant honeycombing sign: prominent honeycomb-like cyst formation occupying more than 70% of the areas of the lung affected by fibrosis

Two leading societies have published criteria for the diagnosis of UIP based on HRCT findings:

In patients with UIP, areas of ground-glass attenuation tend to increase in extent or progress to fibrosis despite treatment 8,13.  In those with more active inflammation involving the pulmonary interstitium, there is a faster progression of honeycombing in long-term follow-up 10. The average rate of progression of honeycombing in patients with idiopathic usual interstitial pneumonia according to one study was 0.4% of lung volume per month 7.

A key imaging differential on cross-sectional imaging would be:

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Cases and figures

  • Figure 1: pathology - histology of UIP
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  • Case 1: classic example
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  • Case 2: on radiograph
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  • Case 3: with emphysema
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  • Case 4
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  • Case 5
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  • Case 6
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  • Case 7
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  • Case 8
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  • Case 9
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  • Case 10
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  • Case 11
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  • Case 12
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  • Case 13
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  • Case 14
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  • Case 15
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  • Case 16: with a single lung transplant
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  • Case 17
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  • Case 18
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  • Case 19
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  • Case 20
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