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Cryptogenic bilateral fibrosing pleuritis.
D R Buchanan1, I D Johnston, I H Kerr
1Brompton Hospital, London.
Summary
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.
- 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.