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Nephrol Dial Transplant (1994) 9: 1477-1481
© 1994 European Renal Association-European Dialysis and Transplant Association

case-report

Renal disease in POEMS syndrome: report on a case and review of the literature

G. J. Navis1,, R. P. F. Dullaart1, E. Vellenga1, J. D. Elema2 and P. E de Jong1

1Department of Internal Medicine Groningen, The Netherlands 2Department of pathology State University Hospital Groningen, The Netherlands

Correspondence and offprint requests to: Correspondence and offprint requests to: G. J. Navis MD, Department of Internal Medicine, State University Hospital, Oostersingel 59, 9713 EZ Groningen, The Netherlands

POEMS syndrome is a multisystem disorder associated with plasma cell dyscrasias. This report describes a patient with POEMS-associated renal disease and reviews the literature on biopsy-proven renal involvement in POEMS syndrome. Our patient had glomerulonephritis with membranoproliferative features on light-microscopy without characteristic findings on immunofluorescence, and with ultra- structural evidence of glomerular microangiopathy. Ultrastructural evidence of microangiopathy was also found in vasa nervorum. In 20 other cases of POEMS- associated renal disease, 16 had glomerular disease. Light-microscopy showed membranoproliferative-like glomerulopathy in 14 patients and glomerular microan giopathy in two. Ultrastructural evidence of microangi opathy was present in all 15 patients in whom electron- microscopy was done. Thus, in most patients with POEMS-associated glomerular disease a characteristic lesion is present with evidence of endothelial injury. As endothelial damage is also found in endoneural vessels, generalized endothelial injury may play a role in non-renal manifestations of POEMS syndrome. In previous reviews manifestations of the POEMS syndrome were similar for patients with or without myeloma. Among patients with biopsy-proven glomerular disease, however, myeloma patients are underrepres ented. Whether this represents a sampling error or has true pathophysiological significance remains to be established.

Keywords: case report; POEMS syndrome; renal disease; review


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