A patient with POEMS syndrome: the pathology of glomerular microangiopathy.

[systemic capillary leak syndrome]

POEMS syndrome (polyneuropathy , organomegaly , endocrinopathy , monoclonal gammopathy and skin changes ) is potentially fatal multisystem disorder but its renal complication has often been overlooked because renal involvement is not necessarily included in the diagnostic criteria of POEMS . This report documents the patient with POEMS syndrome with long -term renal histopathological changes evaluated by renal biopsy . A 32 -year -old Japanese woman presented with symptoms consistent with POEMS syndrome associated with proteinuria and IgA-λ type monoclonal gammopathy . Initial renal biopsy for confirmation of diagnosis revealed the proliferation of glomerular capillary loops located in the expanded mesangial matrices associated with glomerular enlargement . Electron microscopy examination of the renal biopsy revealed the presence of double contoured glomerular basement membrane containing peculiar fibrillary structure . The patient was therefore initially diagnosed as membranoproliferative glomerulonephritis (MPGN )-like lesion without any significant immunoglobulins and complements deposition . The patient was subsequently admitted to hospital on five occasions due to renal dysfunction and anasarca for the next four years of her clinical course . The severity of anasarca was correlated mainly with serum titer of vascular endothelial growth factor (VEGF ) during this period . Acute renal failure occurred at the last admission and the second biopsy was performed . An increased mesangial matrix and frequent global sclerosis of the glomeruli with arteriolosclerosis was noted in this second biopsy compared to the first one . These findings of renal biopsies suggest that the glomerular microangiopathy of POEMS syndrome may occur in the context of systemic capillary leak syndrome superimposed on chronic endothelial injury .