Cryptogenic Fibrosing Pleuritis
  • Alessandra Anna Manco
    Department of Medicine and Surgery, Respiratory Disease and Lung Function Unit, University of Parma, Parma, Italy
  • Veronica Alfieri
    Department of Medicine and Surgery, Respiratory Disease and Lung Function Unit, University of Parma, Parma, Italy
  • Letizia Gnetti
    Pathology Unit, University Hospital of Parma, Parma, Italy
  • Marina Aiello
    Department of Medicine and Surgery, Respiratory Disease and Lung Function Unit, University of Parma, Parma, Italy
  • Giuseppina Bertorelli
    Department of Medicine and Surgery, Respiratory Disease and Lung Function Unit, University of Parma, Parma, Italy
  • Maria Majori
    Department of Cardiothoracic and Vascular Diseases, Pneumology and Endothoracic Endoscopy Unit, University Hospital of Parma, Parma, Italy
  • Alfredo Chetta
    Department of Medicine and Surgery, Respiratory Disease and Lung Function Unit, University of Parma

Keywords

Cryptogenic fibrosing pleuritis, pleural thickening, pleural effusion

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.

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References

  • Huggins JT, Sahn SA. Causes and management of pleural fibrosis.Respirology 2004;9:441–447. doi:10.1111/j.1440-1843.2004.00630.x
  • Azoulay E, Paugam B, Heymann MF, Kambouchner M, Haloun A, Valeyre D, et al. Familial extensive idiopathic bilateral pleural fibrosis. Eur Respir J 1999;14:971–973. doi:10.1034/j.1399-3003.1999.14d41.x
  • Buchanan DR, Johnston IDA, Kerr IH, Hetzel MR, Corrin B, Turner-Warwick M. Cryptogenetic bilateral fibrosing pleuritis. Br J Dis Chest 1988;82:186. doi:10.1016/0007-0971(88)90042-3
  • Hayes JP, Wiggins J, Ward K, Muldowney F, FitzGerald MX. Familial cryptogenic fibrosing pleuritis with Fanconi’s syndrome (renal tubular acidosis). A new syndrome. Chest 1995;107:576–578. doi:10.1378/chest.107.2.576
  • Lee-Chiong Jr TL, Hilbert J. Extensive idiopathic benign bilateral asynchronous pleural fibrosis. Chest 1996;109:564–565. doi:10.1378/chest.109.2.564
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    Published: 2021-05-17
    Issue: 2021: Vol 8 No 5 (view)


    How to cite:
    1.
    Manco AA, Alfieri V, Gnetti L, Aiello M, Bertorelli G, Majori M, Chetta A. Cryptogenic Fibrosing Pleuritis. EJCRIM 2021;8 doi:10.12890/2021_002498.