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

POEMS syndrome is a multisystem disorder associated with plasma cell d yscrasias. 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 membranopr oliferative features on light-microscopy without characteristic findin gs on immunofluorescence, and with ultrastructural evidence of glomeru lar microangiopathy. Ultrastructural evidence of microangiopathy was a lso found in vasa nervorum. In 20 other cases of POEMS-associated rena l disease, 16 had glomerular disease. Light-microscopy showed membrano proliferative-like glomerulopathy in 14 patients and glomerular microa ngiopathy in two. Ultrastructural evidence of microangiopathy was pres ent in all 15 patients in whom electronmicroscopy was done. Thus, in m ost patients with POEMS-associated glomerular disease a charactertisti c lesion is present with evidence of endothelial injury. As endothelia l 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-prove n glomerular disease, however, myeloma patients are underrepresented. Whether this represents a sampling error or has true pathophysiologica l significance remains to be established.