Low-density lipoprotein apheresis for PLA2R-related membranous glomerulonephritis accompanied by IgG4-related tubulointerstitial nephritis.
Aged
Biopsy
Blood Component Removal
/ methods
Glomerulonephritis, Membranous
/ complications
Humans
Immunoglobulin G
/ blood
Immunoglobulin G4-Related Disease
/ complications
Kidney
/ pathology
Lipoproteins, LDL
/ metabolism
Male
Microscopy, Fluorescence
/ methods
Nephritis, Interstitial
/ complications
Nephrotic Syndrome
/ complications
Parotitis
/ diagnosis
Receptors, Phospholipase A2
/ metabolism
Steroids
/ administration & dosage
Treatment Outcome
IgG4-related kidney disease
LDL apheresis
Membranous glomerulonephritis
PLA2R
Tubulointerstitial nephritis
Journal
CEN case reports
ISSN: 2192-4449
Titre abrégé: CEN Case Rep
Pays: Japan
ID NLM: 101636244
Informations de publication
Date de publication:
11 2020
11 2020
Historique:
received:
12
03
2020
accepted:
06
06
2020
pubmed:
20
6
2020
medline:
7
9
2021
entrez:
20
6
2020
Statut:
ppublish
Résumé
IgG4-related disease preferentially involves the kidney by tubulointerstitial nephritis with IgG4-positive plasma cell filtration and/or membranous glomerulonephritis. We reported the case of a 68-year-old man with IgG4-related tubulointerstitial nephritis combined with antiphospholipase A2 receptor (PLA2R)-related membranous glomerulonephritis, in which distinguishing between idiopathic PLA2R-related and IgG4-related secondary membranous glomerulonephritis was difficult. We diagnosed him as having IgG4-related disease, based on a serum IgG4 level of 170 mg/dL and the presence of IgG4-related parotiditis. On renal biopsy, there was tubulointerstitial nephritis with IgG4-positive plasma cell filtration, which was compatible with IgG4-related disease and membranous glomerulonephritis, with concomitant positive staining for PLA2R on immunofluorescence microscopy. The renal function immediately recovered after steroid treatment, probably because of the improvement in the tubulointerstitial lesions, but his nephrotic syndrome was steroid-resistant. Low-density lipoprotein (LDL) apheresis therapy was effective for membranous glomerulonephritis and increased his serum albumin from 1.4 to 2.8 g/dL. Although IgG4-related kidney disease usually accompanies secondary membranous glomerulonephritis, the positive PLA2R staining suggested a concomitant primary membranous glomerulonephritis. The recent treatment strategy, including LDL apheresis, for primary and secondary membranous glomerulonephritis was discussed briefly in this report.
Identifiants
pubmed: 32557252
doi: 10.1007/s13730-020-00494-6
pii: 10.1007/s13730-020-00494-6
pmc: PMC7502100
doi:
Substances chimiques
Immunoglobulin G
0
Lipoproteins, LDL
0
PLA2R1 protein, human
0
Receptors, Phospholipase A2
0
Steroids
0
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
395-403Références
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