Acquired partial lipodystrophy and C3 glomerulopathy: Dysregulation of the complement system as a common pathogenic mechanism.
Nefrologia 2017 Dec 23. Epub 2017 Dec 23.
Unidad de Inmunología, Hospital Universitario La Paz, IdiPAZ, Madrid, España; Centro de Investigación Biomédica en Red (CIBERER U754), Madrid, España; Universidad Autónoma de Madrid, Madrid, España. Electronic address:
The activation of the alternative pathway of the complement is involved in the development of several renal diseases, such as atypical haemolytic uremic syndrome and C3 glomerulopathy. In C3 glomerulopathy, a high percentage of patients have circulating levels of the autoantibody called C3NeF, which causes systemic dysregulation of the complement system. In some cases, the presence of this antibody has been related with abnormalities of adipose tissue, causing acquired partial lipodystrophy (Barraquer-Simons syndrome). Read More