2021
DOI: 10.1016/j.xkme.2021.03.013
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Primary Membranous Nephropathy With Enhanced Staining of Exostosin 1/Exostosin 2 in the Glomeruli: A Report of 2 Cases

Abstract: Technological advances have allowed the discovery of 6 subtypes of membranous nephropathy based on target antigens: M-type phospholipase A 2 receptor (PLA 2 R), thrombospondin type 1 domain-containing 7A (THSD7A), neural epidermal growth factor-like 1 protein, semaphorin 3B, exostosin 1 (EXT1), and EXT2. EXT1/EXT2 are thought to be associated with secondary (autoimmune) membranous nephropathy. Although it has been reported that PLA 2 R-and THSD7A-associated membranous nephropathy have rarely been detected conc… Show more

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Cited by 12 publications
(7 citation statements)
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“…Neither our study nor the studies of Sethi and coworkers (9) found exostosin positive in patients with primary PLA2R membranous nephropathy. However, two patients with primary membranous nephropathy were found to be exostosin positive in a recent report (16), suggesting that the relationship between exostosin and membranous nephropathy needs to be further studied.…”
Section: Discussionmentioning
confidence: 96%
“…Neither our study nor the studies of Sethi and coworkers (9) found exostosin positive in patients with primary PLA2R membranous nephropathy. However, two patients with primary membranous nephropathy were found to be exostosin positive in a recent report (16), suggesting that the relationship between exostosin and membranous nephropathy needs to be further studied.…”
Section: Discussionmentioning
confidence: 96%
“…This case presents a unique scenario of PLA2r-associated MN with EXT1 positivity, unreported in original or follow-up studies [ 2 , 3 ]. Nevertheless, a single case of PLA2r-positive membranous nephropathy with EXT1/EXT2 positivity has been documented, with only one out of 39 PLA2r-associated MN samples testing positive for EXT1/ EXT2 [ 6 ]. A study by Iwakura et al suggested that EXT1/EXT2-associated MN may occur independently of autoimmune diseases, proposing it as a potential marker for autoimmune disease occurrences in the future [ 7 ].…”
Section: Discussionmentioning
confidence: 99%
“…Our results suggested that Ext1/Ext2 could occur without the presence of autoimmune diseases. Interestingly, our study included two patients with Ext1/Ext2-associated MN that showed positive staining for other podocyte antigens 31 . Further case accumulation is needed to clarify the roles and features of Ext1/Ext2-associated MN.…”
Section: Discussionmentioning
confidence: 99%