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Published on: October 30, 2010

[An unusual cause of chylothorax: primary amyloidosis]

G Jeannin1, H Marson, P Merle

  • 1Service de pneumologie, Hôpital Gabriel Montpied, CHU de Clermont-Ferrand, France. gjeannin@chu clermontferrand.fr

Revue Des Maladies Respiratoires
|June 7, 2008
PubMed
Abstract

Insights

Primary amyloidosis can present as chylothorax, a rare pleural effusion. Endomyocardial biopsy confirmed AL amyloidosis, highlighting proBNP

Area of Science:

  • Cardiology
  • Pulmonology
  • Hematology

Background:

  • Amyloidosis involves extracellular protein deposits, rarely causing pleural effusion (6%).
  • Clinical signs of pleural amyloidosis are often non-specific, complicating diagnosis.

Observation:

  • A 77-year-old man presented with anasarca and recurrent pleural effusions.
  • Pleural fluid analysis revealed chylothorax; echocardiography showed restrictive cardiomyopathy.
  • Endomyocardial biopsy confirmed AL amyloidosis after negative peripheral biopsies.

Findings:

  • This case demonstrates primary amyloidosis manifesting as chylothorax.
  • High proBNP levels (24000 ng/l) were observed.
  • Endomyocardial biopsy is crucial for diagnosing systemic amyloidosis.

Implications:

  • Pleural effusions in amyloidosis are multifactorial, leading to delayed diagnosis and critical illness.
  • Early diagnosis and treatment are vital, given the poor prognosis (median survival 2 months).
  • ProBNP serves as a valuable prognostic marker in amyloidosis.

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