Disappearance of intraglomerular lipoprotein thrombi and marked improvement of nephrotic syndrome by bezafibrate treatment in a patient with lipoprotein glomerulopathy.
Lipoprotein glomerulopathy (LPG) is a hereditary disorder characterized by intraglomerular lipoprotein thrombi and increased serum apolipoprotein (apo) E. Patients with LPG usually manifest with nephrotic syndrome, and some progress to renal failure; however, no effective therapeutic regimen has been established for this disease. We experienced a patient with LPG for whom bezafibrate treatment was very effective. This 30-year-old Japanese woman had nephrotic syndrome and type III hyperlipoproteinemia. Renal biopsy showed markedly dilated capillary lumina containing massive lipoprotein thrombi. Plasma apo E concentration was elevated to twice that of normal controls. She was proved to be a heterozygote of apo E2 Kyoto (Arg25Cys). After 2 years treatment with bezafibrate (400 mg/day), her plasma albumin gradually increased from 2.1 to 4.0 mg/dl, and intraglomerular lipoprotein thrombi disappeared almost completely. Bezafibrate decreased plasma apo E and dramatically increased high density lipoprotein (HDL)-cholesterol. The decrease in apo E was observed mainly in the pre-beta-fraction, not in the alpha fraction. Lipidological analyses of our patient suggest that the origin her lipoprotein thrombi may be mainly from pre-beta-lipoproteins and that HDL might be involved in resolving lipoprotein thrombi. Our case suggests that administration of fibrates such as bezafibrate may be a novel therapeutic strategy for resolving intraglomerular thrombi and improving nephrotic syndrome in patients with LPG.
Arai
Takeshi
T
Department of Internal Medicine, Ashiya Municipal Hospital, 39-1 Asahigaoka, Ashiya, Hyogo 659-0012, Japan.
Yamashita
Shizuya
S
Yamane
Mitsukazu
M
Manabe
Noriko
N
Matsuzaki
Toshiyuki
T
Kiriyama
Kazuo
K
Kanayama
Yoshio
Y
Himeno
Seiichi
S
Matsuzawa
Yuji
Y
D000328:Adult; D053329:Apolipoprotein E2; D001057:Apolipoproteins E / Q000097:blood / Q000235:genetics; D001629:Bezafibrate / Q000627:therapeutic use*; D008076:Cholesterol, HDL / Q000097:blood; D005260:Female; D053442:High-Density Lipoproteins, Pre-beta; D006801:Humans; D000960:Hypolipidemic Agents / Q000627:therapeutic use*; D007674:Kidney Diseases / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D007678:Kidney Glomerulus / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D008074:Lipoproteins / Q000032:analysis*; D008075:Lipoproteins, HDL / Q000097:blood; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000209:etiology; D013927:Thrombosis / Q000188:drug therapy* / Q000378:metabolism
D053329:Apolipoprotein E2; D001057:Apolipoproteins E; D008076:Cholesterol, HDL; D053442:High-Density Lipoproteins, Pre-beta; D000960:Hypolipidemic Agents; D008074:Lipoproteins; D008075:Lipoproteins, HDL; D001629:Bezafibrate