Lipoprotein glomerulopathy: first case in a white European

Abstract

Since 1988, 11 cases of a new entity, 'Lipoprotein glomerulopathy' (LG), were described in Japan. Some of these reports suggested that this glomerular lipid storage is due to excess apo E associated with heterozygous E2/3 apo E isoform. We report the first case of LG in a white European with no such lipid abnormalities. Proteinuria was discovered in 1967 when he was 42. Blood pressure and renal function were normal. Family history was negative. Renal biopsy disclosed lesions which were only understood at the time of the Japanese publications. They were composed of endocapillary glomerular deposits. Staining for lipids disclosed capillary loop obstruction with lipid droplets. Electron microscopy showed confluent droplets of various sizes obstructing capillary loops. Proteinuria progressively increased. In 1974 repeat renal biopsy showed the same lipid deposits, now associated with focal-segmental glomerulosclerosis (FSGS). Several serum lipoprotein and apolipoprotein studies ruled out any specific lipid derangement. This suggested a local glomerular disorder, presumably affecting the glomerular endocapillary disposal of lipids. A third biopsy showed progressive glomerular destruction by FSGS with persistence of the lipid droplets. Renal insufficiency progressed and haemodialysis was started in 1992. This observation suggests that LG is a local glomerular, not a general lipid disorder and indicates that this disease is not restricted to Asian patients.