Membranous nephropathy is typically classified as idiopathic and secondary, but nowadays the number of atypical membranous nephropathy (aMN) is increasing, many of which cannot determine its etiology in China. In this study, we compared the clinical and pathological characteristics of idiopathic membranous nephropathy (iMN) with aMN with unknown etiology from a single center in China.We retrospectively reviewed the clinical data of 577 patients with iMN and aMN at Peking University People's Hospital from January 2006 to December 2015 over a 10-year period, and analyzed their clinical and pathological characteristics. The level of serum phospholipase A2 receptors (PLA2R) antibody was detected in 106 iMN and 162 aMN patients.There were 278 iMN patients and 299 aMN patients who were included into this study in 3210 cases of renal biopsy during a 10-year period in our hospital. The average age of patients with iMN was significantly older than those with aMN (54.77 ± 13.01 vs 47.13 ± 16.16, P < .001). Around 75 patients (27%) were smokers in iMN patients, and 111 patients (37.1%) in aMN patients (P = .009). The mainly clinical manifestation of these 2 groups was nephrotic syndrome (61.5% in iMN group vs 58.4% in aMN group), but there were more patients accompanied with nephritis syndrome in aMN group than iMN group (17.1% vs 6.1%, P < .001). The immunofluorescence of renal biopsy showed “full house” in aMN group; and IgG subclass of the glomeruli demonstrated IgG4 (90.4%) was commonest in iMN group, but IgG1 (94.6%) in aMN group. 51 (48.1%) patients with iMN were detected positive PLA2R antibody in their serum, and 93 (57.4%) in aMN patients (P = .168). The patients with positive PLA2R antibody had higher positive rate of microscopic hematuria and urinary protein, lower albumin.The aMN patients are younger, higher smoking rate, its main clinical manifestation is nephrotic syndrome, but more of them accompanied with nephritis syndrome than those in iMN patients. Serum PLA2R antibody could not distinguish aMN from iMN. aMN could be a special glomerular disease in China, and need a further research on a larger scale.
Glomeruli instance segmentation from pathologic images is a fundamental step in the automatic analysis of renal biopsies. Glomerular histologic manifestations vary widely among diseases and cases, and several special staining methods are necessary for pathologic diagnosis. A robust model is needed to segment and classify glomeruli with different staining methods and apply in cases with various glomerular pathologic changes. Herein, pathologic images from renal biopsy slides stained with three basic special staining methods were used to build the data sets. The snapshot group included 1970 glomeruli from 516 patients, and the whole-slide image group included 8665 glomeruli from 148 patients. Cascade Mask region-based convolutional neural net architecture was trained to detect, classify, and segment glomeruli into three categories: i) GN, structural normal; ii) global sclerosis; and iii) glomerular with other lesions. In the snapshot group, total glomeruli, GN, global sclerosis, and glomerular with other lesions achieved an F1 score of 0.914, 0.896, 0.681, and 0.756, respectively, which were comparable with those in the whole-slide image group (0.940, 0.839, 0.806, and 0.753, respectively). Among the three categories, GN achieved the best instance segmentation effect in both groups, as determined by average precision, average recall, F1 score, and Mask mean Intersection over Union. The present model segments and classifies multistained glomeruli with efficiency and robustness. It can be applied as the first step for more detailed glomerular histologic analysis.
Background IgG4-related disease (IgG4-RD) often affects multiple organs and tissues, especially the kidneys, and is characterized by interstitial nephritis, obstructive nephropathy, and in rare cases glomerulopathy (including membranous nephropathy). Case presentation In this article, we report a patient with nephrotic syndrome as the only initial manifestation. Membranous nephropathy was confirmed by renal biopsy, but without any renal interstitial lesions. The nephrotic syndrome completely resolved after treatment with immunosuppressants but recurred after drug withdrawal, which was accompanied by acute kidney injury. Ultimately, IgG4-related interstitial nephritis with membranous nephropathy was confirmed by a second renal biopsy. After routine administration of steroids and cyclophosphamide, renal function returned to normal after 2 months, and nephrotic syndrome was ameliorated after 5 months. Conclusion Special attention should be paid to this rare condition in the clinical setting. In patients with membranous nephropathy (MN) that is accompanied by multi-system damage, impaired renal function, elevated IgG4 levels (absolute or relative value), negative PLA2R, and/or renal interstitial plasma cell infiltration, the possibility of IgG4-related kidney disease (IgG4-RKD) should be carefully assessed.
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