Contributed by Valerie Lyons, M.D. and Robert Kelly, Ph.D.
Published on line in July 1996
A 45 year old woman developed proteinuria and a skin rash during her sixth pregnancy in 1986. Positive serologies included a speckled anti-nuclear antibody, anti-sjögrens syndrome-a (ro) and a low titer rheumatoid factor. She has been negative for anti-double stranded DNA, anti-smith, anti-sjögrens syndrome-b (la) and anti-rheumatoid nuclear protein throughout the six year course of her disease. Her lupus was complicated by a capillary leak syndrome, resulting in edema. She frequently had vasculitic rashes which usually responded to short doses of prednisone. However, in the spring of 1993, she developed severe proteinuria 9.4 grams/24 hour, refractory to prednisone therapy. Her renal function did not change and remained stable with a creatinine of 1.4-1.5 mg%, creatinine clearance 105 ml/min. Her plasma complement C3 and C4 levels, which had been persistently low, increased rapidly during the spring and summer (C3=1.09 g/l and C4=0.42 g/l), and became supernormal by september ( C3=4.35 g/l and C4=0.93 g/l). Due to the severity and refractory nature of her proteinuria, she was admitted to the university of pittsburgh medical center in the fall of 1993 for elective renal biopsy. Percutaneous biopsy of the kidney revealed membranoproliferative glomerulonephritis, type I, and focal interstitial nephritis by light microscopy. On electron microscopy, a representative glomerulus revealed marked hypercellular loops with obliterated lumina. Peripheral capillary walls and mesangium contained subendothelial dense deposits which by immunofluorescence stained with IgM, IgG, lamda and kappa chains. In December 1993, she was started on cytoxan pulse therapy. A bone marrow biopsy in the spring of 1994 showed evidence of Waldenstrom's Macroglobulinemia.