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. 2021 May 17;8(5):002498.
doi: 10.12890/2021_002498. eCollection 2021.

Cryptogenic Fibrosing Pleuritis

Alessandra Manco  1 , Veronica Alfieri  1 , Letizia Gnetti  2 , Marina Aiello  1 , Giuseppina Bertorelli  1 , Maria Majori  3 , Alfredo Chetta  1
Affiliations

Cryptogenic Fibrosing Pleuritis

Alessandra Manco et al. Eur J Case Rep Intern Med. .

Abstract

We report the case of a 46-year-old male patient who was referred for chest pain and bilateral pleural effusion. Despite treatment with antibiotics and steroids, the pleural effusion worsened over a few months until pulmonary function was halved. The CT scan showed bilateral pleural thickening with right basal opacity. Histology revealed extensive fibrotic tissue with focal collections of lymphocytes and giant cells without traces of asbestos bodies. Since no evidence of an infectious, embolic or occupational aetiology was found, this bilateral pleural effusion progressing to diffuse pleural thickening was diagnosed as cryptogenic fibrosing pleuritis, a rare pleural disease.

Learning points: Bilateral pleural effusion progressing to diffuse pleural thickening was diagnosed as cryptogenic fibrosing pleuritis, a rare pleural disease.Cryptogenic fibrosing pleuritis was treated with high-dose corticosteroids.The patient showed stable disease at 6-year follow-up.

Keywords: Cryptogenic fibrosing pleuritis; pleural effusion; pleural thickening.

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Conflict of interest statement

Conflicts of Interests: The authors declare there are no competing interests.

Figures

Figure 1
Figure 1
Imaging in 2013. (A) Chest x-ray from February 2013 showing bilateral pleural thickening with minimal bilateral pleural effusion, most evident on the right side. (B) Chest CT scan from February 2013 showing the presence of parenchymal basal consolidations, bilaterally associated with pleural effusion with initial signs of bilateral pleural thickening of a maximum thickness of about 20 mm
Figure 2
Figure 2
Pleural biopsy histological findings. (A) Extended fibrotic tissue with focal reactive mesothelium. The pleural surface is covered by organizing fibrin. (B) Focal collections of lymphocytes with fibrous tissue intermingled on the subpleural fat. The alveolar spaces are trapped by fibrosis; in some areas foreign-body-type giant cell granulomatous inflammation can be seen
Figure 3
Figure 3
Lung function progression: the graph shows the progressive decline in lung function and the subsequent stability since 2016 up to 2019
Figure 4
Figure 4
Radiological evaluation. (A) Chest x-ray from September 2016 showing a right basal consolidation attributable to a round atelectasis and associated with bilateral pleural effusion. (B) Chest CT scan from July 2018 showing stable radiological findings, confirming the right pleural thickening
See this image and copyright information in PMC

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