RENAL INVOLVEMENT IN POEMS SYNDROME

We described a patient with a POEMS syndrome (Polyneuropathy, Organome galy, Endocrinopathy, Monoclonal grammapathy and Skin changes) who was found to have renal involvement with peculiar renal pathological find ings. Hitherto, 17 other cases, most of them from Japan, of POEMS synd rome with renal involvement, have been published. Clinical features ar e variable: acute renal failure with anasarca or moderate chronic rena l insufficiency with mild proteinuria. This latter presentation often passes unnoticed. There is no severe HTA, no microangiopathic hemolyti c anemia. Renal biopsy shows prominent glomerular changes which are un usual and distinct from membranoproliferative glomerulonephritis (MPGN ) and from glomerular thrombotic microangiopathy (TMA). Mesangial prol iferation and thickening of the capillary wall with double contour evo ke by light microscopy a MPGN. By immunofluorescent microscopy. no imm unoglobulins or complement deposits are found. The finding of mesangio lytic lesions has led to the term of << mesangiolytic glomerulonephrit is >>. The presence, on electron microscopy, of lucent subendothelial space could evoke TMA. But there is neither thrombi, nor arteriolar ch anges. We are inclined to presume that microangiopathic lesions are du e to chronic injury of glomerular endothelial cells, exacerbated at ou tbreaks of the disease. An increased production of IL-6 could support the efficacity of corticosteroid therapy, particularly in acute clinic al situations.