Abstract: TH-PO622
A Case of Lipoprotein Glomerulopathy Which Achieved Remission by Bezafibrate Monotherapy
Session Information
- Fellows/Residents Case Reports: Genetic Diseases, Pregnancy, Monoclonal Gammopathy
November 02, 2017 | Location: Hall H, Morial Convention Center
Abstract Time: 10:00 AM - 10:00 AM
Category: Nephrology Education
- 1302 Fellows and Residents Case Reports
Authors
- Kanda, Manabu, Japan Community Health care Organization Sendai Hospital, Sendai, Japan
- Sato, Mitsuhiro, Japan Community Health care Organization Sendai Hospital, Sendai, Japan
- Sanada, Satoru, Japan Community Health Care Organization Sendai Hospital, Sendai, Japan
- Sato, Hiroshi, Clinical Pharamacology and Therapeutics, Graduate School of pharmaceutical Sciences, Tohoku University, Sendai, Japan
- Sato, Toshinobu, Japan Community Health care Organization Sendai Hospital, Sendai, Japan
- Taguma, Yoshio, Japan Community Health care Organization Sendai Hospital, Sendai, Miyagi, Japan
Background
Lipoprotein glomerulopathy (LPG) is a rare hereditary renal disease with a relatively rapid progression to renal failure, accompany with proteinuria, hyperlipidemia, and lipoprotein thrombi in glomeruli. The first LPG case was reported in 1989, then our facility firstly reported efficacy of intensive therapy with combination of three lipid-lowering agents, which decreased proteinuria by removal of lipoprotein thrombi in 2000. In this report, we show that even fibrate monotherapy could ameliorate proteinuria in patients with LPG.
Methods
A 38-year-old Japanese man was admitted with a 10-year history of proteinuria. None of his family has renal disease or dyslipidemia. His body mass index was 17.8, and blood pressure was 140/90 mmHg. He has no corneal opacity, xanthoma nor leg edema. Laboratory tests showed urine protein 1.30 g/day, serum creatinine 0.80 mg/dL, total cholesterol 155 mg/dL, and the triglyceride 142mg/dL. Apolipoprotein profiles revealed B 66 mg/dL, normal range 73 to 109, and E 11.9 mg/dL, 2.7 to 4.3. Heterozygous ApoE-Sendai mutation was detected by DNA sequencing analysis.
Renal Biopsy showed distinct dilatation of glomerular capillary lumina filled with lipoprotein thrombi which was positive for oil red O and apoE staining (figure).
Bezafibrate monotherapy decreased proteinuria to less than 0.3 g/day in five months. Clinical remission has been lasted for 2 years, though the ApoE level was still high.
Conclusion
We experienced a LPG case without dyslipidemia and family history. The previous reports have shown the effectiveness of combination therapy multiple lipid-lowering agents, however, in our case even monotherapy with fibrate could decrease proteinuria and prevent disease progression.