Cryptogenic Fibrosing Pleuritis

Our plain-English summary of Cryptogenic Fibrosing Pleuritis, by A. Manco, V. Alfieri, L. Gnetti, et al (European Journal of Case Reports in Internal Medicine, 2021).

The study

• Authors: A. Manco, V. Alfieri, L. Gnetti, M. Aiello, G. Bertorelli, M. Majori, A. Chetta
• Journal: European Journal of Case Reports in Internal Medicine
• Published: 2021;8(5):002498
• Cited by: 1 article, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Unlabelled: 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.

© EFIM 2021.

In plain English

This case report described a 46-year-old man who developed chest pain in October 2012 and fluid around the lungs (pleural effusion) that did not improve with short courses of antibiotics and steroids. When referred to the authors' unit in Parma, Italy, in February 2013, the patient had thickening of the lining around both lungs (pleural thickening) and a moderately severe restrictive breathing pattern. Tests for infection and rheumatic disease were negative and no cancer cells were found. A biopsy of the pleura showed dense fibrous scar tissue with mild inflammation and no asbestos bodies. The patient denied asbestos exposure. Because no asbestos bodies were found either, the authors stated they could exclude asbestos-related disease. The condition was diagnosed as cryptogenic fibrosing pleuritis, meaning pleural scarring of unknown cause. The patient received high-dose prednisone (0.7 mg/kg for 6 weeks, then tapered over 6 months). Lung function declined at first, but there was no further worsening after September 2016. The authors reported that the patient was stable at 6-year follow-up and concluded that the best treatment for this rare condition has not been established.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (single patient)
• Who was studied: One 46-year-old man, referred to a respiratory unit at the University of Parma, Italy, in February 2013 and followed up from 2013 to 2019
• Exposure: No asbestos exposure was identified. The patient denied environmental exposure to asbestos and denied exposure to organic powders or chemicals, and no asbestos bodies were found in the pleural biopsy.
• Main results:
  • The parietal pleural biopsy showed dense collagen fibrosis, mild non-specific lymphocytic inflammation and no asbestos bodies
  • The authors excluded known causes of pleural effusion, including asbestos-related diseases
  • The patient was treated with high-dose corticosteroids
  • Lung function declined after treatment, but there was no further functional or radiological worsening from September 2016 onward
  • At 6-year follow-up the patient was functionally and radiologically stable
• The authors' conclusion: The authors concluded that very few cases of cryptogenic fibrosing pleuritis are known, that the treatment approach has not been properly established, and that further studies are needed.

Topics

Medical terms in this study, explained in our Lexicon: Bilateral, Pleural Effusion, CT Scan, Pleural Thickening, Histology, Asbestos Bodies, Diffuse Pleural Thickening.

Related studies

• British Thoracic Society study of cryptogenic fibrosing alveolitis: current presentation and initial management. Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society — I.D. Johnston, R.J. Prescott, et al. (1997)
• Cryptogenic fibrosing alveolitis and lung cancer: the BTS study — J.M. Harris, I.D. Johnston, et al. (2010)
• Fine structural changes in cryptogenic fibrosing alveolitis and asbestosis — B. Corrin, A. Dewar, et al. (1985)
• How useful is GLUT-1 in differentiating mesothelial hyperplasia and fibrosing pleuritis from epithelioid and sarcomatoid mesotheliomas? An international collaborative study — A.N. Husain, M.K. Mirza, et al. (2014)
• Fibrosing pleuritis with extrathoracic extension — B.I. Hoffbrand, M.R. Hetzel, et al. (1985)
• An outbreak of pleural mesothelioma and chronic fibrosing pleurisy in the village of Karain/Urgüp in Anatolia — Y.I. Baris, A.A. Sahin, et al. (1978)

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