Progressive massive fibrosis

Case contributed by Dr Chris Cadman

Presentation

This 67 year-old man presented with cough and minimal breathlessness on climbing hills. He was a past smoker of 20 years but quit smoking 40 years ago. He is retired now but previously worked as a tunneller (drilling tunnels to create sewage pipes).

Patient Data

Age: 67
Gender: Male
Modality: X-ray

There is extensive nodularity throughout the lungs, with more confluent opacification within the right midzone and left lower zone, upper zone and apices.

Modality: CT

There are diffuse, dense tiny nodules throughout the lungs in keeping with industrial dust exposure. There are dense conglomerates of consolidation within the upper lobes, consistent with progressive massive fibrosis. There is patchy consolidation and nodular change within the right middle and lower lobes and the left lower lobe, suggesting a possible super-added infection. There is interlobular septal thickening within the lower lobes but no other features to suggest fluid overload. There is small volume, peripherally calcified hilar and mediastinal lymphadenopathy. There are bilateral calcified pleural plaques indicative of previous asbestos exposure.

Case Discussion

Pneumoconiosis is the deposition of industrial dust within the lung, most commonly coal dust (coal worker's pneumoconiois - CWP) or silicon dioxide (silicosis). Pneumoconiosis predisposes to chronic bronchitis and emphysema and patients may present as such. There is also an increased risk of lung cancer and pulmonary infection.

On chest radiographs, pneumoconiosis demonstrates tiny nodular opacities, with the density of the nodules dependent on the causative particles. On CT, the nodular opacities are more apparent, with a centrilobar or subpleural location. There may be mediastinal and hilar lymphadenopathy with eggshell calcification.

Progressive massive fibrosis (PMF) represents the coalescence of larger nodules, resulting in mass-like opacities that may be seen on plain radiographs and CT. They typically occur in the posterior aspects of the upper lobes and may be associated with fibrosis and parenchymal distortion. These masses migrate centrally towards the hilar over time, leaving cicatricial emphysema behind. PMF occurs more commonly in silicosis, compared with CWP.

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Case Information

rID: 51851
Case created: 10th Mar 2017
Last edited: 11th Mar 2017
System: Chest
Inclusion in quiz mode: Included

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