Usual interstitial pneumonia

Case contributed by Dr Prashant Mudgal

Presentation

Elderly male with chronic dyspnoea and impaired PFT (restrictive pattern). Heavy smoker.

Patient Data

Age: 75 years
Gender: Male
X-ray

Reticular and linear pulmonary opacification is noted in the peripheral subpleural regions with basal predominance bilaterally, findings are most likely suggestive of pulmonary interstitial fibrosis.

CT

There is lung architectural distortion due to patchy areas of intralobular septal thickening resulting in reticular pattern in peripheral and subpleural lung regions noted asymmetrically in bilateral lung fields with basal predominance. 
 
There is evidence of dilated and distorted centrilobular bronchioles within the areas of reticulations suggestive of traction bronchiolectasis. There are surrounding areas of fibrosis with segmental and subsegmental bronchiectasis. There are multiple subpleural lines visible suggestive of fibrosis. 
 
In addition to above findings there is evidence of patchy areas of concentric macrocystic honeycombing with peripheral and subpleural and  in distribution seen asymmetrically in both lungs with lower zone and posterior predominance.

Few reactive and calcified mediastinal lymph nodes noted the largest measures 13x11 mm (precarinal).

Mild apical fibrotic pleural thickening noted bilaterally.

Lung volume loss and architectural distortion with reticular pattern, traction bronchiolectasis and bronchiectasis and fibrosis and subpleural lines with concentric macrocystic honeycombing in asymmetrically bilateral patchy subpleural and peripheral distribution as well as few areas of ground glass attenuation are findings most likely suggestive of idiopathic interstitial fibrosis or usual interstitial pneumonia (UIP).

Case Discussion

Idiopathic pulmonary fibrosis is more common in smokers. The disease has a poor prognosis with median survival of 2 to 3 years. 

Diagnostic criteria include:

Major criteria

  • exclusion of known cause of pulmonary fibrosis
  • restrictive pattern in pulmonary function test
  • bibasilar reticulation with minimal ground glass opacity
  • negative bronchial lavage report for another disease

Minor criteria

  • age >50 years
  • dyspnoea on exertion with insidious onset
  • duration of illness>3 months
  • Velcro Like respiratory crackles
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Case information

rID: 39199
Case created: 25th Aug 2015
Last edited: 29th Nov 2016
System: Chest
Tag: pm
Inclusion in quiz mode: Included

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