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Cryptogenic bilateral fibrosing pleuritis.

D R Buchanan1, I D Johnston, I H Kerr

  • 1Brompton Hospital, London.

British Journal of Diseases of the Chest
|April 1, 1988
PubMed
Summary
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We identified a new condition, cryptogenic bilateral fibrosing pleuritis, characterized by unexplained pleural thickening. This condition differs from asbestos-related disease and may respond to corticosteroids.

Area of Science:

  • Pulmonology
  • Pathology

Background:

  • Bilateral pleural effusions can progress to diffuse pleural thickening.
  • Distinguishing this from asbestos-related disease is crucial.

Purpose of the Study:

  • To describe a novel idiopathic condition of bilateral pleural thickening.
  • To propose the term "cryptogenic bilateral fibrosing pleuritis" (CBFP).

Main Methods:

  • Case series of four patients with idiopathic bilateral pleural thickening.
  • Clinical, radiographic, CT, and histological evaluation.
  • HLA B44 typing and asbestos fiber analysis.

Main Results:

  • Patients presented with illness, chest pain, dyspnea, cough, malaise, pleural effusions, and radiographic shadowing.

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  • CT confirmed bilateral extensive pleural thickening.
  • Histology revealed fibrous thickening without asbestos bodies.
  • All patients were HLA B44 positive.

    • Conclusions:

    • CBFP is a distinct entity from asbestos-related pleural disease.
    • Surgical decortication was effective in three patients.
    • Corticosteroids showed partial efficacy in managing contralateral disease.