Intravascular cardiac lipoproteinosis: extrarenal manifestation of lipoprotein glomerulopathy

Morris, Catherine S.; Bois, Melanie C.; Aust, Charles H.; Thomas, Ryan M.; Sethi, Sanjeev; Maleszewski, Joseph J.

doi:10.1016/j.carpath.2019.04.006

Cited by 3 publications

(4 citation statements)

References 26 publications

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“…In addition, unexplained splenomegaly has been reported in patients with LPG (11,12). Morris et al (13) also reported that intravascular cardiac lipoproteinosis may be an extrarenal manifestation of LPG. However, the exact pathophysiologic mechanism of these extrarenal manifestations remains unclear.…”

Section: Discussionmentioning

confidence: 98%

Case Report: A Pediatric Case of Lipoprotein Glomerulopathy in China and Literature Review

et al. 2021

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Background: Lipoprotein glomerulopathy is a rare kidney disease characterized by lipoprotein thrombi in the glomerulus. Here, we report a case of lipoprotein glomerulopathy in a Chinese pediatric patient. Furthermore, we summarized the clinical features and genetic characteristics of lipoprotein glomerulopathy in China.Case Presentation: An 11-year-old Chinese girl presented with nephrotic syndrome with anemia (98 g/L). After excluding secondary causes, primary nephrotic syndrome was considered. Treatment with prednisone (60 mg/day) did not improve her condition. Renal biopsy showed marked dilation of the capillary lumen with lipoprotein thrombi and positive oil red O staining. Genetic testing revealed the genetic variant c.127C > T (p.R43C), known as the Kyoto mutation of the APOE gene. These findings are consistent with the diagnosis of lipoprotein glomerulopathy. Prednisone was gradually tapered and captopril was initiated. A 2-year follow-up revealed elevated urine protein and serum creatinine levels. We also reviewed 17 pediatric and 156 adult cases of lipoprotein glomerulopathy reported in China from the year of creation to 2021. The most common clinical features were edema, hematuria, hypertriglyceridemia, and increased serum apoE levels. Extra-renal manifestations included anemia, splenomegaly, and cardiac lipoprotein deposition.Conclusion: APOE Kyoto is the most common mutation in patients with lipoprotein glomerulopathy. In China, homozygosity for E3 (E3/3) is the most common isoform.

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Section: Discussionmentioning

confidence: 98%

Case Report: A Pediatric Case of Lipoprotein Glomerulopathy in China and Literature Review

et al. 2021

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“…LPG can affect any age group. 3 It is usually found in patients of East Asian descent, with only 18 cases identified in patients of non-Asian descent, 2 , 10 , 12 , 20 , 22 , 23 , 25–33 of which only 9 include confirmatory gene sequencing: 1 APOE Chicago, 22 3 APOE Kyoto, 12 , 23 1 APOE Las Vegas, 25 2 APOE Modena, 27 , 29 and 2 APOE Osaka/Kurashiki. 10 It has a male predilection and usually presents with proteinuria found incidentally on routine laboratory testing.…”

Section: Discussionmentioning

confidence: 99%

Lipoprotein Glomerulopathy, First Case Report from Canada

Ting¹,

McRae²,

Schwartz³

et al. 2022

IJNRD

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Lipoprotein glomerulopathy (LPG) is caused by a mutation in the apolipoprotein E gene ( APOE ) gene and is characterized by lipoprotein thrombi in glomerular capillaries. Here, we describe a case of LPG, the first to be reported from Canada and the first case of LPG in North America to be associated with the APOE Tokyo/Maebashi mutation (p.Leu162_Lys164del, traditional nomenclature 142_144del). A 49-year-old man of Chinese descent with a previous diagnosis of dyslipidemia and a new diagnosis of hypertension was found to have proteinuria on routine urinalysis. Renal biopsy showed markedly dilated glomerular capillaries filled with pale staining mesh-like material that stained positive for Oil-Red-O, consistent with lipoprotein thrombi. APOE gene sequencing confirmed the diagnosis of LPG. The patient was treated with fenofibrate and perindopril. His lipid profile normalized and proteinuria dropped to minimal levels. Repeat renal biopsy 2 years after the first showed resolution of lipoprotein thrombi but with rare residual granular densities by electron microscopy consistent with lipoprotein in the subendothelial space, supporting the hypothesis that this subendothelial material contains precursors to lipoprotein thrombi.

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“…However, there is no evidence of a direct link between LPG and these diseases. The only related extrarenal manifestation of LPG that has been confirmed is intravascular coagulation (110). It was reported that a 50-year-old white man had severe proteinuria with high lipid levels and a kidney pathology of LPG.…”

Section: Extrarenal Manifestations and Other Complicationsmentioning

confidence: 99%

“…The most typical pathological appearance of LPG under light microscopy is the dilatation of the glomerular capillary lumen filled with eosinophilic granular and vacuolar thrombosis (Figure 7). The thrombus is positive for oil red O or Sudan red and negative for silver or PAS staining, indicating the presence of a lipid component in the thrombus (14,22,(110)(111)(112). Using immune-electron microscopy, it has been found that lipids are surrounded by apoE and that the thromboid material is composed of lipoproteins (113).…”

Section: Pathological Manifestations In the Kidneymentioning

confidence: 99%

An Updated Review and Meta Analysis of Lipoprotein Glomerulopathy

Liu

et al. 2022

Front. Med.

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More than 200 cases of lipoprotein glomerulopathy (LPG) have been reported since it was first discovered 30 years ago. Although relatively rare, LPG is clinically an important cause of nephrotic syndrome and end-stage renal disease. Mutations in the APOE gene are the leading cause of LPG. APOE mutations are an important determinant of lipid profiles and cardiovascular health in the population and can precipitate dysbetalipoproteinemia and glomerulopathy. Apolipoprotein E-related glomerular disorders include APOE2 homozygote glomerulopathy and LPG with heterozygous APOE mutations. In recent years, there has been a rapid increase in the number of LPG case reports and some progress in research into the mechanism and animal models of LPG. We consequently need to update recent epidemiological studies and the molecular mechanisms of LPG. This endeavor may help us not only to diagnose and treat LPG in a more personized manner but also to better understand the potential relationship between lipids and the kidney.

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Intravascular cardiac lipoproteinosis: extrarenal manifestation of lipoprotein glomerulopathy

Cited by 3 publications

References 26 publications

Case Report: A Pediatric Case of Lipoprotein Glomerulopathy in China and Literature Review

Case Report: A Pediatric Case of Lipoprotein Glomerulopathy in China and Literature Review

Lipoprotein Glomerulopathy, First Case Report from Canada

An Updated Review and Meta Analysis of Lipoprotein Glomerulopathy

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