Lipoprotein glomerulopathy is a pathological condition characterized by lipid accumulation in the glomerular capillaries that has been associated with the presence of rare mutants of apolipoprotein E (ApoE). We describe a 51-year-old Italian patient presenting Type III hyperlipidemia and proteinuria in whom renal biopsy showed capillary ectasia and intraluminal lipid deposits, suggesting the diagnosis of lipoprotein glomerulopathy. The patient, who had elevated plasma ApoE level, was found to be heterozygous for a mutation in ApoE (Arg150Cys), designated apoEMODENA. This mutation induces the formation of ApoE dimers that are detectable under non-reducing conditions. Treatment with hypolipidemic drugs did not result in a complete remission of the proteinuria and was accompanied by a slow but progressive worsening of renal function with the persistence of intracapillary lipid thrombi. The introduction of low-density lipoprotein aphaeresis combined with a more aggressive lipid lowering and antihypertensive therapy resulted in the remission of proteinuria and a substantial improvement of renal function. Switching from low-density lipoprotein aphaeresis to plasma filtration did not result in an equivalent control of renal damage. The patient died of intracranial hemorrhage during an acute episode of malignant hypertension.

Lipoprotein glomerulopathy associated with a mutation in apolipoprotein e / Magistroni, Riccardo; Bertolotti, Marco; Furci, Luciana; Fano, Rita Adriana; Leonelli, Marco; Pisciotta, Livia; Pellegrini, Elisa; Calabresi, Laura; Bertolini, Stefano; Calandra, Sebastiano. - In: CLINICAL MEDICINE INSIGHTS. CASE REPORTS. - ISSN 1179-5476. - STAMPA. - 6:(2013), pp. 189-196. [10.4137/CCRep.S12209]

Lipoprotein glomerulopathy associated with a mutation in apolipoprotein e

MAGISTRONI, Riccardo;BERTOLOTTI, Marco;FANO, Rita Adriana;PELLEGRINI, Elisa;
2013

Abstract

Lipoprotein glomerulopathy is a pathological condition characterized by lipid accumulation in the glomerular capillaries that has been associated with the presence of rare mutants of apolipoprotein E (ApoE). We describe a 51-year-old Italian patient presenting Type III hyperlipidemia and proteinuria in whom renal biopsy showed capillary ectasia and intraluminal lipid deposits, suggesting the diagnosis of lipoprotein glomerulopathy. The patient, who had elevated plasma ApoE level, was found to be heterozygous for a mutation in ApoE (Arg150Cys), designated apoEMODENA. This mutation induces the formation of ApoE dimers that are detectable under non-reducing conditions. Treatment with hypolipidemic drugs did not result in a complete remission of the proteinuria and was accompanied by a slow but progressive worsening of renal function with the persistence of intracapillary lipid thrombi. The introduction of low-density lipoprotein aphaeresis combined with a more aggressive lipid lowering and antihypertensive therapy resulted in the remission of proteinuria and a substantial improvement of renal function. Switching from low-density lipoprotein aphaeresis to plasma filtration did not result in an equivalent control of renal damage. The patient died of intracranial hemorrhage during an acute episode of malignant hypertension.
2013
6
189
196
Lipoprotein glomerulopathy associated with a mutation in apolipoprotein e / Magistroni, Riccardo; Bertolotti, Marco; Furci, Luciana; Fano, Rita Adriana; Leonelli, Marco; Pisciotta, Livia; Pellegrini, Elisa; Calabresi, Laura; Bertolini, Stefano; Calandra, Sebastiano. - In: CLINICAL MEDICINE INSIGHTS. CASE REPORTS. - ISSN 1179-5476. - STAMPA. - 6:(2013), pp. 189-196. [10.4137/CCRep.S12209]
Magistroni, Riccardo; Bertolotti, Marco; Furci, Luciana; Fano, Rita Adriana; Leonelli, Marco; Pisciotta, Livia; Pellegrini, Elisa; Calabresi, Laura; B...espandi
File in questo prodotto:
File Dimensione Formato  
Magistroni-2013-Lipoprotein glomerulopathy ass.pdf

Open access

Tipologia: Versione pubblicata dall'editore
Dimensione 2.53 MB
Formato Adobe PDF
2.53 MB Adobe PDF Visualizza/Apri
Pubblicazioni consigliate

Licenza Creative Commons
I metadati presenti in IRIS UNIMORE sono rilasciati con licenza Creative Commons CC0 1.0 Universal, mentre i file delle pubblicazioni sono rilasciati con licenza Attribuzione 4.0 Internazionale (CC BY 4.0), salvo diversa indicazione.
In caso di violazione di copyright, contattare Supporto Iris

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11380/1061735
Citazioni
  • ???jsp.display-item.citation.pmc??? 8
  • Scopus 11
  • ???jsp.display-item.citation.isi??? ND
social impact