Progressive kidney disease in three sisters with elevated lipoprotein(a)

Lipoprotein(a) [Lp(a)] is an atherogenic, LDL-like particle of unknown physiologic function. Elevated serum concentrations of Lp(a) have been linked conceptually and epidemiologically to cardiovascular disease [1–3], but their relationship to the progression of renal disease remains much less clear, despite several hypothesized mechanisms by which this could occur [4–12]. We present a unique case of three sisters with profound elevations in serum Lp(a), progressive but unexplained impairment of kidney function, and renal biopsies demonstrating benign nephrosclerosis and apolipoprotein(a) [apo(a)] deposition.