RENAL-DISEASE IN POEMS SYNDROME - REPORT ON A CASE AND REVIEW OF THE LITERATURE

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 ultrastructural 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 microangiopathy in two. Ultrastructural evidence of microangiopathy was present in all 15 patients in whom electronmicroscopy was done. Thus, in most patients with POEMS-associated glomerular disease a charactertistic 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 underrepresented. Whether this represents a sampling error or has true pathophysiological significance remains to be established