[Partial lipodystrophy, hypocomplementemia and glomerulonephritis].
Renal involvement is found in 20 to 50% of cases of Partial lipodystrophy (PLD). We report 8 cases of PLD of which 6 had a glomerular nephropathy and 2 had no renal disease but all had persistent hypocomplementemia and 5 had circulating nephritic factor (C3NeF). The analysis of these cases and of all the cases reported in the literature shows the specificity of the glomerular involvement. Membranoproliferative glomerulonephritis (MPGN) with dense intramembranous deposits is a constant finding in PLD with renal involvement. This variety of MPGN is well known for being associated with persistent hypocomplementemia. However, the presence of hypocomplementemia and C3NeF in patients with PLD but without nephritis raises the question of the interrelationship between alternative pathway complement activation and the development of MPGN with or without lipodystrophy. There is no valid explanation in the present state of knowledge for the association of partial lipodystrophy hypocomplementemia, and MPGN. From the answer to this problem should emerge a better understanding of the role of complement in renal disease and in particular in the unusual form of glomerular injury seen in MPGN.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003172:Complement C1 / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D003178:Complement C3 Nephritic Factor / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D003182:Complement C5 / Q000032:analysis; D003165:Complement System Proteins / Q000032:analysis*; D005260:Female; D005921:Glomerulonephritis / Q000209:etiology / Q000276:immunology* / Q000473:pathology; D006801:Humans; D008060:Lipodystrophy / Q000150:complications / Q000276:immunology*; D008297:Male
D003172:Complement C1; D003176:Complement C3; D003178:Complement C3 Nephritic Factor; D003181:Complement C4; D003182:Complement C5; D003165:Complement System Proteins
Lipodystrophie partielle, hypocomplementemie et glomerulonephrite.