BackgroundThe recently suggested distinct pathogenic pathways for myeloperoxidase (MPO) and proteinase 3 (PR3) anti-neutrophilic cytoplasmic antibodies (ANCA) associated vasculitis could result in different modes of presentation and outcome. Moreover, kidney outcome was related to a new histopathologic classification of pauci-immune glomerulonephritis. As reports were not always concordant, possible because differences in severity of organ lesions and ethnicity, we evaluated the outcome of a cohort of Central-East European patients with crescentic glomerulonephritis in relation with ANCA specificity and histopathological classification.MethodsSeventy-five patients were consecutively diagnosed by kidney biopsy (76 % MPO-ANCA specificity, 52 % crescentic) and followed for a median period of 3.2 years. Study end-points were response to therapy, end stage renal disease (ESRD) and death.ResultsPR3-ANCA patients were younger, in higher proportion male and had higher Birmingham Vasculitis Activity Scores (BVAS). The kidney disease was severe at presentation (median creatinine 5 mg/dL; 27 % required temporary dialysis) and worst in PR3-ANCA positive patients (50 % patients needed temporary dialysis vs. 19 %). The lung was the second most affected organ (31 % severe lung hemorrhage). Lung and kidney damage were related; the odds of hemorrhagic alveolitis in patients needing dialysis at presentation were 4 (95 % CI 1–13; p = 0.006) times higher than in those who did not. The rate of response to therapy (without signs of active vasculitis and stable or declining serum creatinine) was 60 % and was associated with dialysis independency, older age and higher platelet number at presentation. The probabilities to survival 1 and 5 years for kidney and patient were 93 and 64 %, and respectively 88 and 67 %. Kidney survival was predicted by response to therapy and dialysis independency at presentation. Patients with BVAS < 15 and responding to induction therapy had better chances of survival. Neither response to therapy nor outcome was influenced by ANCA specificity or by the histopathological class.ConclusionsWhen kidney damage is severe in ANCA vasculitis, the need of dialysis at presentation and the response to induction therapy overcome the prognostic utility of both ANCA specificity and histopathological class.
FederationIntroduction and Aims: Loss of podocytes in primary glomerulopathies is crucial for glomerulosclerosis progression which leads to end-stage renal failure in such patients. The mechanism of direct replacement of injured podocytes does not exist so the only way to compensate the integrity of glomerulus is change of cells shape to cover the glomerular tuft with a smaller number of podocytes. Foot process effacement is the typical morphological sign of podocyte respond to stress. Podocyte detachment (PD) from glomerular basement membrane (GBM) develops when podocyte hypertrophy is unsufficient. The aim of investigation was estimation of relationship between range of foot process width (FPW), PD and level of daily proteinuria in patients with primary variants of glomerulopathies. Methods: 42 patients with biopsy proven primary glomerulopathies were included in the study. According to the the results of light and electron microscopy 17 (40,5%) patients had membranous nephropathy, 8 (19,0%) -focal segmental glomerulosclerosis, 12 (28,6%) -minimal change disease and 5 (11,9%) -proliferative variants of glomerulonephritis (2-IgA-nephropathy, 3 -membrano-proliferative glomerulonephritis). Standart laboratory and instrumental investigations were perfomed. Samples of serum and urine were obtained in the day of byopsy. FPW and PD were measured using Image J software (NIH, 1997). FPW was counted as ratio of GBM length to amount of foot processes in every electronogramm using correction factor π/4 as described in previous works. PD was calculated as percentage of bare areas of GBM. Results: There were no statistically significant differences between FPW and PD in patients with different forms of glomerulopathies ( p>0,05). There was negative correlation between 31, p<0,05). Daily proteinuria rate positively correlated with FPW (r=0,52, p<0,05) while inverted relation with level of PD was found (r=-0,36, p<0,05). The same pattern was detected comparing groups of patients with and without nephrotic syndrome. The level of daily proteinuria was higher in patients with more expressed hyaline droplet degeneration of tubular epithelial cells. Conclusions: Unlike data published in recent works we found no difference of FPW and PD rate in patients with different forms of glomerulonephritis. Strong positive correlation of FPW with proteinuria range confirms the role of podocytes in development of high proteinuria and nephrotic syndrome, considering that there were no abnormalities in tubular reabsorbtion of protein. Interestingly the detachment of podocytes from GBM does not increase proteinuria range, more over inverse relationship was detected. Probably this fact can be explained by unknown mechanisms of transcellular transport of protein rather than directly through bare parts of GBM. Nagoya University Graduate School of Medicine, Nagoya, Japan Introduction and Aims: The clinicopathological characteristics of PLA2R-related membranous nephropathy (MN) in Japan remain unclear. Methods: We studied retrospectively the outcomes ...
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.