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Pleural effusions in systemic amyloidosis.

John L Berk1

  • 1The Pulmonary and Amyloid Treatment and Research Program, Department of Medicine, Boston University School of Medicine, Massachusetts 02118-2526, USA. jberk@lung.bumc.bu.edu

Current Opinion in Pulmonary Medicine
|June 2, 2005
PubMed
Summary
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Large pleural effusions in systemic amyloidosis are rare, primarily affecting primary systemic amyloidosis patients. Direct amyloid infiltration of the pleura causes these effusions, not heart or kidney issues.

Area of Science:

  • Pulmonary Medicine
  • Cardiology
  • Nephrology

Background:

  • Large, recurrent pleural effusions are rare but challenging complications in systemic amyloidoses.
  • Primary systemic amyloidosis is the predominant form associated with these effusions.

Purpose of the Study:

  • To investigate the mechanisms of pleural effusion formation and persistence in systemic amyloidoses.
  • To clarify the role of cardiomyopathy and nephrotic syndrome in these effusions.

Main Methods:

  • Retrospective analysis of 35 primary systemic amyloidosis patients with large, refractory pleural effusions.
  • Comparison of demographics, pleural fluid, echocardiographic, and renal function data with 120 primary systemic amyloidosis cardiomyopathy patients without effusions.

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Main Results:

  • Amyloid infiltration of the pleural surface is the primary cause of exudative pleural effusions.
  • Neither cardiomyopathy nor nephrotic syndrome adequately explained the effusions.
  • Median survival for untreated patients with effusions was 1.6 months.

Conclusions:

  • Direct pleural infiltration by amyloid is the main driver of large pleural effusions in primary systemic amyloidosis.
  • Cardiomyopathy contributes but is insufficient to cause and sustain these effusions.
  • Other forms of systemic amyloidosis do not typically cause clinically significant pleural effusions.