Crescentic C3 glomerulopathy with acquired partial lipodystrophy: An unusual cause of rapidly progressive renal failure.

Complement component C3 glomerulopathy (C3GP) is a recently defined entity characterized by predominant glomerular C3 fragment deposition with absent or scanty immunoglobulin deposition due to abnormal control of complement activation, deposition, or degradation.[1] C3GP is subcategorized morphologically into dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) based on electron microscopic (EM) findings. DDD previously called membranoproliferative glomerulonephritis Type II refers to cases with hyperosmiophilic, dense transformation of glomerular basement membranes (GBM) on EM[2] and other cases of C3GP without the pathognomonic ultrastructural findings are designated C3GN. DDD has an estimated prevalence of 2–3/million population[3] and traditionally presents with nephritic-nephrotic syndrome in children or young adults.[4] We describe a rare case of DDD with crescentic glomerulonephritis, presenting as rapidly progressive renal failure (RPRF) in an adult female patient, associated with acquired partial lipodystrophy (Barraquer Simons’ or Dunnigan-Koeberling syndrome).[5]