2013
DOI: 10.1007/s10067-013-2406-0
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IgG4-related membranous nephropathy with high blood and low urine IgG4/IgG ratio: a case report and review of the literature

Abstract: Membranous nephropathy (MN) is a rare manifestation of IgG4-related disease. Interestingly, the significance of IgG4 has also been documented in idiopathic MN (IMN). Previous studies reported that urine IgG4/IgG ratios were significantly higher in IMN compared with other kinds of nephropathy, indicating that impairment of charge selectivity barrier seemed to be an obvious characteristic of IMN. Although high blood concentration of IgG4 is very common in IgG4-related MN, no study about the urine IgG4 has been d… Show more

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Cited by 11 publications
(5 citation statements)
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“…We tend to favor the latter explanation, at least in the cases of PLA 2 R-associated MN that have positive hepatitis serology or cancer. Interestingly, although MN has been described in several cases of IgG4-related disease (57), this was not associated with PLA 2 R deposits, and anti-PLA 2 R was not detected in such cases of MN (58)(59)(60) or in cases of IgG4-related disease without MN (61).…”
Section: Figurementioning
confidence: 89%
“…We tend to favor the latter explanation, at least in the cases of PLA 2 R-associated MN that have positive hepatitis serology or cancer. Interestingly, although MN has been described in several cases of IgG4-related disease (57), this was not associated with PLA 2 R deposits, and anti-PLA 2 R was not detected in such cases of MN (58)(59)(60) or in cases of IgG4-related disease without MN (61).…”
Section: Figurementioning
confidence: 89%
“…However, in glomerular diseases, urinary loss of IgG subclasses is proportional to the serum levels [43], which could rule out the possibility of selective excretion of IgG subclasses as a reason for the proportional increase of IgG4 in IMN. Some studies even suggest that IgG4 molecules in very high serum levels may bind with 1 another or with other molecules, slowing down filtration and increasing the serum concentration [44]. As serum levels of IgG4 did not differ significantly among the groups in the present study, the filtration hypothesis was minimized; thus, the increased serum %IgG4 in IMN became a relevant fact.…”
Section: Resultsmentioning
confidence: 66%
“…The dominance of IgG4 deposition is more believed to favor iMN than the secondary forms of MN, as seen in MN secondary to malignancies [13]. Furthermore, as stipulated in Xiao's study in 2013, that "an increase in the urine samples with IgG4 is indicative of membranous nephropathy among other related diseases", further affirms the role of IgG4 in MN [14]. It is believed that dysregulated IgG4 play a role in the development of iMN.…”
Section: Discussionmentioning
confidence: 93%