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Nephrol Dial Transplant (2002) 17: 492-494
© 2002 European Renal Association-European Dialysis and Transplant Association

Case Report

An unusual evolution of the systemic capillary leak syndrome

Cécile Vigneau, Jean-Philippe Haymann, Noujoud Khoury, Jean-Daniel Sraer and Eric Rondeau

Service de Néphrologie A, Hôpital Tenon, Association Claude Bernard, Paris, France

Keywords: amyloidosis; Clarkson disease; systemic capillary leak syndrome



   Introduction
 
Systemic capillary leak syndrome (SCLS) is characterized by sudden shock with diffuse oedema sparing the lungs [1]. Currently, about 50 cases have been reported with a 5-year survival of 25% [2,3].

For the first time, we describe a case of SCLS evolving over 20 years and culminating in a late and fatal myeloma with amyloidosis.



   Case
 
A 33-year-old black male, was referred to our department in 1981 for the following symptoms consistent with typical SCLS: several episodes of shock with generalized oedema; diffuse pain; and transient anuria. A monoclonal gammapathy of undetermined significance (MGUS) was discovered at that time. Three years later, he had minimal proteinuria (monoclonal component). Femoral artery superficialization was performed to obtain a permanent and easy vascular access, and the patient was treated during subsequent crises by colloid or . . . [Full Text of this Article]



   Discussion
 


   Acknowledgments
 


   Notes
 


   References
 

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