A Rare Case of Polyneuropathy and Monoclonalgammopathy with Recurrent Acute Kidney Injury

Abstract

POEMS syndrome is a rare paraneoplastic syndrome and there are few reports of polyneuropathy and monoclonal gammopathy associated with kidney dysfunction. Here, we report a case of POEMS syndrome with recurrent acute kidney injury (AKI). A 52-year-old man presented with bilateral aggravating paresthesia and latermotor weakness of the lower extremities accompanied by repeated elevation of serum creatinine. The patient was finally diagnosed with POEMS syndrome on the basis of fulfilling the two mandatory major criteria (polyneuropathy and monoclonal gammopathy), one other major criterion (sclerotic bone lesion), and several minor criteria. A renal biopsy was performed to clarify the cause of AKI and showed membranoproliferative glomerulonephritis-like lesions with mesangiolysis and endothelial cell injury. This case illustrates that renal manifestations, not included in the diagnostic criteria for POEMS, can be apparent before various other systemic symptoms.

Efficacy of novel agents in patients with nephropathy associated with POEMS syndrome

“…MPGN-like lesions are proliferation of mesangial and endothelial cells with basement membrane thickening and stratification was similar to MPGN by light and electron microscopy. Mesangiolysis is a glomerular process leading to lesions of continuous endothelial cells and glomerular mesangial cells, endothelial cell proliferation, endothelial swelling and subendothelial widening, endothelial injury caused mesangial interposition, finally mesangial proliferation and mesangial sclerosis [12]. Glomerular endothelial cell proliferation was defined as the endothelial cell number was increased along with enlargement of the subendothelial spaces [13], glomerular subendothelial widening was defined as formation of new layer(s) of basal lamina like double contour basement membrane in capillary of the glomerulus [14].…”