Hypocomplementemic proliferative glomerulonephritis with C3 nephritic-factor-like activity in multiple myeloma.
Advanced renal failure, nephrotic-range proteinuria due to proliferative glomerulonephritis and multiple myeloma with circulating IgG2 lambda and free lambda light-chain paraproteins occurred in a 31-year-old male. Commonly established causes of renal failure in multiple myeloma were excluded. Immunofluorescence revealed heavy granular glomerular deposition of C3. Serum C3 was decreased, and C3c was increased. C3 nephritic-factor (C3 NeF)-like activity was demonstrated in the serum. Plasmapheresis and chemotherapy resulted in a decrease in paraprotein concentration up to 90%, a decrease in C3 NeF-like activity to negligible, normal serum complement levels and a marked improvement in both renal function and proteinuria. With reference to the literature, the possibility of a syndrome of paraproteinemia, C3 NeF-like activity and glomerulonephritis is forwarded.
Bourke
E
E
Department of Medicine, Emory University School of Medicine, Atlanta, Ga.
Campbell
W G
WG
Piper
M
M
Check
I J
IJ
D000328:Adult; D003178:Complement C3 Nephritic Factor / Q000032:analysis / Q000097:blood*; D003169:Complement Inactivator Proteins / Q000097:blood*; D015432:Glomerulonephritis, Membranoproliferative / Q000097:blood* / Q000378:metabolism; D006801:Humans; D007678:Kidney Glomerulus / Q000032:analysis; D008297:Male; D009101:Multiple Myeloma / Q000150:complications*
D003178:Complement C3 Nephritic Factor; D003169:Complement Inactivator Proteins