Lipoprotein glomerulopathy treated with LDL-apheresis (Heparin-induced Extracorporeal Lipoprotein Precipitation system): a case report

Abstract

Introduction: Lipoprotein glomerulopathy is a glomerulonephritis which was described for the first time by Saito in 1989 and is currently acknowledged as a separate nosological entity. It is histologically characterized by a marked dilatation of the glomerular capillaries and the presence of lipoprotein thrombi in the glomerular lumens. The dyslipidemic profile is similar to that of type III dyslipoproteinemia with Apolipoprotein E values that are often high; proteinuria and renal dysfunction are present. Proteinuria often does not respond to steroid and cytostatic treatments. The phenotypic expression of lipoprotein glomerulopathy is most probably correlated to a genetic alteration of the lipoprotein metabolism (mutation of the Apolipoprotein E coding gene). In literature, lipoprotein glomerulopathies have mainly been reported in Japanese and Chinese subjects, except for three cases in the Caucasian race, reported in France and the USA.

Case presentation: We describe the case of a 60-year-old female, Caucasian patient suffering from lipoprotein glomerulopathy, carrier of a new mutation on the Apolipoprotein E gene (Apolipoprotein E(MODENA)), and treated successfully with low density lipoprotein-apheresis with the Heparin induced extracorporeal lipoprotein precipitation system. After a first phase of therapeutic protocol with statins, the patient was admitted for nephrotic syndrome, renal failure and hypertension. Since conventional treatment alone was not able to control dyslipidemia, aphaeretic treatment with heparin-induced Extracorporeal Lipoprotein Precipitation - apheresis (HELP-apheresis) was started to maintain angiotensin converting enzyme inhibitor therapy for the treatment of hypertension. Treatment with HELP-apheresis led to a complete remission of the proteinuria in a very short time (four months), as well as control of hypercholesterolemia and renal function recovery.

Conclusion: According to this case of lipoprotein glomerulopathy, we believe that renal damage expressed by proteinuria correlates to the levels of lipids and, furthermore, the treatment with HELP-apheresis, by lowering low-density lipoprotein cholesterol and triglycerides, may be considered as a therapeutic option in synergy with pharmacological treatment in the treatment of lipoprotein glomerulopathy.

Figure 1

Lipids and Fibrinogen values before every single H.E.L.P.-apheresis (first ten months of treatment). After the first ten months of apheretic treatment the mean values were: Total Cholesterol from 243 mg/dl to 184 mg/dl, HDL-Cholesterol from 65 mg/dl to 77 mg/dl, LDL-Cholesterol from 178 mg/dl to 106 mg/dl, Triglycerides from 285 mg/dl to 161 mg/dl, Fibrinogen from 405 mg/dl to 378 mg/dl, Lp(a) from 61 mg/dl to 51 mg/dl.

Figure 2

Creatinine and Urine Protein to Creatinine Ratio before every H.E.L.P.-apheresis (first ten months of treatment). After the first ten months of apheretic treatment the mean values were: Creatinine from 1.9 mg/dl to 1.5 mg/dl and Urine Protein to Creatinine Ratio from 3.3 to 0.13 (protein and creatinine are expressed in mg/dl).