Partial lipodystrophy, mesangiocapillary glomerulonephritis, and complement dysregulation. An autoimmune phenomenon.
Partial lypodistrophy (PLD) is a rare disease in which, there is loss of fat usually from the upper part of the body. The disease is frequently associated with mesangiocapillary (membranoproliferative) glomerulonephritis Type II (MCGN II). In the early 1970s, it was noticed that MCGN II and/or PLD was sometimes associated with dysfunction of the complement system as reported in several case descriptions and studies. Subsequently, an IgG autoantibody was detected-C3 nephritic factor (C3NeF). The target of this autoantibody is the alternative pathway C3 convertase-C3bBb. There are sporadic case reports that linked PLD, MCGNII, and C3NeF with autoimmune diseases. This association may be more than a coincidence. The complement deficiency may lead to perturbation of the immune system, which may trigger some of the autoimmune diseases. This article will be focused on the association among PLD, MCGN II and C3NeF.
Levy
Y
Y
Department of Medicine B, Sheba Medical Center, Tel-Hashomer, Israel.
George
J
J
Yona
E
E
Shoenfeld
Y
Y
D001327:Autoimmune Diseases / Q000150:complications; D015551:Autoimmunity; D003178:Complement C3 Nephritic Factor / Q000276:immunology*; D003170:Complement Pathway, Alternative; D003165:Complement System Proteins / Q000276:immunology*; D015432:Glomerulonephritis, Membranoproliferative / Q000150:complications / Q000276:immunology*; D006801:Humans; D008060:Lipodystrophy / Q000150:complications / Q000276:immunology*
D003178:Complement C3 Nephritic Factor; D003165:Complement System Proteins