Unusual glomerulopathy with atypical thickening of the glomerular basement membrane and intramembranous microparticles

Nazneen, Arifa; Nakashima, Yumoko; Zha, Yan; Le, Viet Thang; Taguchi, Takashi; Nishioka, Katsuaki; Tadokoro, Masato; Taura, Koichi

doi:10.1007/s10157-008-0103-0

Cited by 6 publications

(3 citation statements)

References 4 publications

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“…Five cases (case 1, 11, 14, 15, and 17) [3], case 18 [12], case 19 [13], case 20 [14], case 21 [15], case 22 [16], case 23 [17], case 24 [18], and case 25 [19], correspond to each reference which has been published in this special issue.…”

Section: Collection Of the Patientsmentioning

confidence: 97%

Proposal of podocytic infolding glomerulopathy as a new disease entity: a review of 25 cases from nationwide research in Japan

et al. 2008

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Section: Collection Of the Patientsmentioning

confidence: 97%

Proposal of podocytic infolding glomerulopathy as a new disease entity: a review of 25 cases from nationwide research in Japan

et al. 2008

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“…We report our institutional experience with a series of MN cases containing MSD deposits. Glomerular deposits with microspherular substructure have been described for decades [9][10][11][19][20][21][22][23][24]. However, the reports seemed somewhat inconsistent in the definition of the term, microspherular, and there was some descriptive overlap with PIG, which has quite distinctive ultrastructural features.…”

Section: Discussionmentioning

confidence: 99%

Characterization of Membranous Nephropathy with Microspherular Deposits

Ren,

Hou

2023

Glomerular Dis

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Introduction: Membranous nephropathy (MN) is a common cause of adult nephrotic syndrome in the US. The typical ultrastructural finding is of global uniformly dense subepithelial electron-dense immune complex deposits along glomerular basement membranes (GBM). However, early reports described deposits with a unique microspherular substructure. There was variability in what was identified as microspherular, sometimes overlapping with other entities such as podocyte infolding glomerulopathy (PIG). Currently, the nature, composition, and clinical significance of these microspherular deposits (MSD) remain unknown. Method: We report the clinicopathologic features of a series of MN cases with MSD, with detailed ultrastructural charactarization as well as PLA2R and THSD7A immunohistochemical and IgG subclass staining characteristics. The proportion of MSD to overall deposits segregated into two groups: global MSD with >50% MSD (n=14) and segmental MSD with <50% (n=5). Results: The size and appearance of the microspherules were nearly identical in global and segmental MSD groups (mean diameter of 77.9 nm and 77.2 nm respectively), with subepithelial (n=19) or intramembranous (n=12) distributions in all cases. Mesangial MSD's (n=5) were only found in the global MSD group. The majority of biopsies (86% of global MSD and 100% of segmental MSD) were Ehrenreich-Churg stage II or above; early stage I was only observed in the global MSD group. All but 3 cases were PLA2R/THSD7A double negative; 1 THSD7A positive in global MSD and 2 PLA2R positive in segmental MSD. IgG1 was the dominant subclass in the global MSD group and IgG4 was dominant in the segmental MSD group, including the 2 PLA2R positive cases. Conclusion: The findings suggest that MSD's are more commonly associated with secondary MN. This case series is the largest to date, and the findings may yield etiologic and prognostic information on this rare but unique subset of MN, and provide a well-characterized cohort of cases for future studies.

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“…Previous reports include 4 cases of PIG complicated by FSGS [4, 5, 6, 7]. One of them, who had serial biopsies, was diagnosed after the first biopsy as having PIG and FSGS associated with vesico-ureteral reflux (VUR) [4].…”

Section: Discussionmentioning

confidence: 99%

A Case of Podocytic Infolding Glomerulopathy with Focal Segmental Glomerulosclerosis

Iguchi

Sohma

Yamazaki

et al. 2013

Case Rep Nephrol Dial

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Podocytic infolding glomerulopathy (PIG) has been proposed as a new disease entity. A 14-year-old girl underwent renal biopsy at our institution because of a chance finding of proteinuria. Light microscopic findings revealed a minor glomerular abnormality, but under a higher magnification, after periodic acid methenamine silver staining, a bubbling appearance in the glomerular basement membrane (GBM) was observed. An electron microscopic examination revealed microspheres in the GBM, which were sparse but global. The patient was diagnosed as having PIG. After 3 years, her urinary protein had increased and a second biopsy was performed, showing focal segmental glomerulosclerosis in addition to a lesser degree of podocytic infolding than at the first biopsy. This is the first report of a case complicated by a different type of glomerulonephritis after being diagnosed as PIG. A few cases of PIG are complicated by focal segmental glomerulosclerosis, suggesting several mechanisms for the disorder.

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Unusual glomerulopathy with atypical thickening of the glomerular basement membrane and intramembranous microparticles

Cited by 6 publications

References 4 publications

Proposal of podocytic infolding glomerulopathy as a new disease entity: a review of 25 cases from nationwide research in Japan

Proposal of podocytic infolding glomerulopathy as a new disease entity: a review of 25 cases from nationwide research in Japan

Characterization of Membranous Nephropathy with Microspherular Deposits

A Case of Podocytic Infolding Glomerulopathy with Focal Segmental Glomerulosclerosis

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