Improvement of nephrotic syndrome by intensive lipid-lowering therapy in a patient with lipoprotein glomerulopathy

Abstract

Lipoprotein glomerulopathy (LPG) is a rare hereditary disease characterized by the accumulation of much thrombi material consisting of lipoproteins at the glomerular capillary lumen. Most patients show nephrotic syndrome; nearly half progress to chronic renal failure. Intensive therapy with lipid-lowering agents reportedly engenders clinical remission with histological resolution. We report the case of a 14-year-old Japanese female patient who had been in a nephrotic condition with hematuria from 4 years old and who had been diagnosed based on pathological and molecular examination at 7 years old. We initially treated the patient with probucol, enalapril (angiotensin-converting enzyme inhibitor: ACEI), and dipyridamole from age 7, but achieved no improvement in her nephrotic status. Subsequently, we replaced probucol with bezafibrate at age 11 and added atorvastatin calcium hydrate and valsartan (angiotensin II receptor blocker: ARB) the following year. The next 3 years' treatment improved her nephrotic status, decreased serum apolipoprotein E, and markedly decreased intraglomerular lipoprotein thrombi. Early and intensive therapy with antilipidemic drugs combined with ACEI and ARB is inferred to be effective for LPG.

Successful treatment of lipoprotein glomerulopathy in a daughter and a mother using niceritrol

“…In the 1990s, probucol was reported to be effective for reducing proteinuria in a patient with early stage LPG who did not exhibit nephrotic syndrome [12]. Later, lipid-lowering therapies, including fibrates, were reported to improve both proteinuria and glomerular lesions in nephrotic LPG patients [4][5][6]13]. Ieiri et al [4] reported that combination therapy with fenofibrate, niceritrol, ethyl-icosapentate and probucol resulted in clinical remission with histologic resolution.…”

“…Kinomura et al [6] reported that intensive combination therapy by adding bezafibrate and ethylicosapentate to statin dramatically decreased proteinuria. Just recently, Matsunaga et al [13] described a patient who had improved nephrotic syndrome after switching from probucol to bezafibrate and atorvastatin, in combination with ACEi and angiotensin II receptor blocker. In contrast, statin monotherapy did not seem to reduce urinary protein excretion, even though a remission of dyslipidemia was achieved [3,11].…”

Five-year follow-up of a case of lipoprotein glomerulopathy with APOE Kyoto mutation

“…Although LPG is known to have a poor renal prognosis, case reports of successful treatment with lipid-lowering therapy have been increasing [17][18][19][20][21][22][23]. Interestingly, the course of the urinary protein level in our patient closely paralleled his TG and cholesterol levels ( Fig.…”

Apolipoprotein E–related glomerular disorders

“…78,98 Given these findings, the prevention of hypertriglyceridemia may be important in LPG treatment, and the efficacy of lipid-lowering agents including fibrates, as shown clinically and by pathology studies, has been reported in several cases in Japan. 66,88,[100][101][102] Additionally, Hu et al 82 compared patient and renal survival rates over 3 years between fenofibrate-treated and control groups, and they confirmed the significant availability of fenofibrate for LPG treatment. In contrast, in apoE2 homozygote glomerulopathy, fibrates have been used in some cases, but the effect is unclear.…”