Hyperuricemia and hypertriglyceridemia indicate tubular atrophy/interstitial fibrosis in patients with IgA nephropathy and membranous nephropathy

2021 ◽  
Author(s):  
Bingman Liu ◽  
Liangyu Zhao ◽  
Qingqing Yang ◽  
Dongqing Zha ◽  
Xiaoyun Si
Keyword(s):  
Iga Nephropathy ◽  
Membranous Nephropathy ◽  
Interstitial Fibrosis ◽  
Tubular Atrophy

scholarly journals Is mycophenolate mofetil combined with low-dose prednisone a treatment option for advanced IgA nephropathy? A 10-year follow-up case and brief literature review

2018 ◽  
Vol 16 ◽  
pp. 205873921880268
Author(s):  
Qijun Wan ◽  
Yongcheng He ◽  
Hongtao Chen ◽  
Hongping Liu ◽  
Saodong Luan ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Iga Nephropathy ◽  
Treatment Option ◽  
Low Dose ◽  
Interstitial Fibrosis ◽  
Immunosuppressive Treatment ◽  
Channel Blockers ◽  
Tubular Atrophy ◽  
Dose Prednisone

IgA nephropathy (IgAN) is now widely recognized as the most common primary glomerulonephritis worldwide, especially in China. The immunosuppressive treatment option for IgAN is still controversial. Previously, we proved that mycophenolate mofetil (MMF; Shanghai Roche, China) combined with low-dose prednisone was an effective and safe option for biopsy-proven mild to moderate IgAN patients in a short term of follow-up. This article we first reported the safety and efficacy of this regimen in a 42-year-old male biopsy-proven advanced 10-year follow-up IgAN case (Lee’s Class V; the patient was biopsied 10 years ago, so the Oxford Mesangial hypercellularity Endocapillary hypercellularity Segmental glomerulosclerosis Tubular atrophy/interstitial fibrosis (MEST) classification was not used). The mycophenolate and prednisone were only given for a limited time. The other main medications included calcium channel blockers and antiplatelet agents. Clinical and laboratory indexes were aperiodic assessed during the 10-year follow-up. The serum creatinine decreased from 356 to around 210 μmol/L and urine excretion protein reduced from 3.4 g/d to about 0.5 g/d after 6 months of the initiation of this regimen, respectively. These perfect treatment effects could maintain well during the whole follow-up period. No obvious complications were observed.

IgA nephropathy: analysis of progression in pediatric patients

2021 ◽  
Vol 25 (3) ◽  
pp. 61-67
Author(s):  
I. A. Kazyra ◽  
А. V. Sukalo
Keyword(s):  
Iga Nephropathy ◽  
B Lymphocyte ◽  
Interstitial Fibrosis ◽  
Prognostic Significance ◽  
Pediatric Nephrology ◽  
Lymphocyte Activation ◽  
Healthy Children ◽  
Tubular Atrophy ◽  
Factors Affecting ◽  
Highly Active

The aim of the study was to analyze the rate of progression of IgA nephropathy (IgAN) in childhood and factors affecting prognosis. The study included 54 children with a morphologically verified diagnosis of IgAN (36 boys, 18 girls) aged 2 to 17 years, who were under observation in the nephrology department of the "2nd Children's City Clinical Hospital" of the National Center for Pediatric Nephrology and Renal Replacement therapy in Minsk in the period from 2013 to 2020. The participation of deGal-IgA1, markers of T- and B-lymphocyte activation, pro-inflammatory and pro-fibrotic molecules in the development of the disease has been shown. AG was registered in 18 of 54 (33,3 %) children, nocturnal AG in 11/43 (23,4 %), signs of cardiac remodeling in 10/49 (20,4 %). A decrease in the level of adiponectin, vitamin D, an increase in obestatin in comparison with healthy children makes it possible to attribute patients with IgAN to the risk group for the development of cardiovascular disorders, which implies the need for timely monitoring and correction. In most cases in childhood IgAN is characterized by a benign course without signs of progression. The prognostic significance of highly active nephritis, impaired renal function at the onset of the disease, T1 (tubular atrophy / interstitial fibrosis in 25–50 %) by MEST, proteinuria over 0,8 g/24 hours as risk factors for progression was shown.

scholarly journals Clinical Relevance of Serum Galactose Deficient IgA1 in Patients with IgA Nephropathy

2020 ◽  
Vol 9 (11) ◽  
pp. 3549
Author(s):  
Jin Sug Kim ◽  
Hyeon Seok Hwang ◽  
Sang Ho Lee ◽  
Yang Gyun Kim ◽  
Ju-Young Moon ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Clinical Relevance ◽  
Interstitial Fibrosis ◽  
Healthy Controls ◽  
Ckd Progression ◽  
Serum Samples ◽  
Tubular Atrophy ◽  
Appropriate Treatment ◽  
Cox Proportional Hazard Models ◽  
New Biomarkers

New biomarkers of IgA nephropathy (IgAN) are needed for non-invasive diagnosis and appropriate treatment. There is emerging evidence that galactose deficient IgA1 (Gd-IgA1) is a pivotal molecule in the pathogenesis of IgAN. However, few studies have investigated the role of Gd-IgA1 as a biomarker in IgAN. In this study, we investigated the clinical relevance of serum Gd-IgA1 levels in patients with IgAN. Two hundred and thirty biopsy-proven IgAN patients, 74 disease controls (patients with non-IgAN nephropathy), and 15 healthy controls were enrolled in this study. Levels of serum Gd-IgA1 were measured using an ELISA kit in serum samples obtained the day of renal biopsy. We compared levels of serum Gd-IgA1 according to the type of glomerular disease and analyzed the association between Gd-IgA1 levels and clinical and pathological parameters in patients with IgAN. We then divided IgAN patients into two groups according to Gd-IgA1 level and investigated the predictive value of Gd-IgA1 for progression of chronic kidney disease (CKD). Serum Gd-IgA1 levels were significantly higher in IgAN patients than disease controls and healthy controls. In patients with IgAN, serum Gd-IA1 levels were significantly correlated with estimated glomerular filtration rate, serum IgA level, and tubular atrophy/interstitial fibrosis. CKD progression was more frequent in IgAN patients with higher serum Gd-IgA1 levels than in those with lower serum Gd-IgA1 levels. Cox proportional hazard models showed that high GdIgA1 level was an independent risk factor for CKD progression after adjusting for several confounders. Our results suggest that serum Gd-IgA1 level is a useful diagnostic and prognostic marker in IgAN patients. Further studies with a larger sample size and longer follow-up duration are needed.

Seropositive PLA2R-associated membranous nephropathy but biopsy-negative PLA2R staining

2020 ◽  
Author(s):  
Jiao Luo ◽  
Wang Zhang ◽  
Cailing Su ◽  
Zhanmei Zhou ◽  
Guobao Wang
Keyword(s):  
Membranous Nephropathy ◽  
Interstitial Fibrosis ◽  
Clinicopathological Characteristics ◽  
Enzyme Linked Immunosorbent Assay ◽  
Double Positive ◽  
Lower Serum ◽  
Tubular Atrophy ◽  
Phospholipase A2 Receptor ◽  
Glomerular Deposits ◽  
Paraffin Tissue

Abstract Background Serum phospholipase A2 receptor (PLA2R) antibody (SAb) and glomerular deposits of PLA2R antigen (GAg) have been tested widely in idiopathic membranous nephropathy (MN). Recently, we noticed a special form of PLA2R-associated MN with positive circulating PLA2R antibody but negative PLA2R deposits in the glomeruli by immunofluorescence on frozen tissue (IF-F). The significance of this form of PLA2R-associated MN is yet to be elucidated. This study aimed to explore the clinicopathological features of these PLA2R-associated MN patients. Methods This study enrolled 229 biopsy-proven PLA2R-associated MN patients with SAb+. SAb was measured by enzyme-linked immunosorbent assay, and GAg was detected by IF-F. These patients were divided into SAb+/GAg+ and SAb+/GAg− groups. Clinicopathological characteristics of SAb+/GAg+ and SAb+/GAg− PLA2R-associated MN patients were compared. PLA2R antigens of 19 SAb+/GAg− PLA2R-associated MN patients were verified by immunohistochemistry on paraffin tissue (IHC-P). Results Among 229 SAb+ PLA2R-associated MN patients, 210 (91.70%) were GAg+ and 19 (8.3%) were GAg−. These 19 SAb+/GAg− PLA2R-associated MN patients presented positive PLA2R deposits by IHC-P. Compared with SAb+/GAg+ PLA2R-associated MN patients, SAb+/GAg- PLA2R-associated MN patients had higher levels of serum PLA2R antibody (P = 0.004), increased proteinuria (P = 0.008), lower serum albumin (P = 0.019), more prominent chronic pathological lesions in terms of glomerulosclerosis score (P = 0.025), interstitial fibrosis score (P = 0.016), tubular atrophy score (P = 0.010) and total renal chronicity score (P = 0.010), and were more likely to be accompanied by focal segmental glomerulosclerosis (P = 0.014). Higher SAb level was associated with the total renal chronicity score (odds ratio per 100 RU/mL, 1.16; 95% confidence interval 1.01–1.33; P = 0.033). Conclusions PLA2R-associated MN patients with seropositive PLA2R antibody but negative PLA2R deposits in the glomeruli by IF-F have higher levels of SAb and worse clinicopathological manifestations compared with their double-positive counterparts. IHC-P can be an alternative technique to reveal PLA2R glomerular deposits.

P0417THE ABSENCE OF C3 DEPOSIT IN MEMBRANOUS NEPHROPATHY IS ASSOCIATED WITH SPONTANEOUS REMISSION

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Cristina Rabasco ◽  
Ana Martínez ◽  
Rosa Ortega ◽  
Mario Espinosa
Keyword(s):  
Membranous Nephropathy ◽  
Interstitial Fibrosis ◽  
Immunosuppressive Treatment ◽  
Spontaneous Remission ◽  
Glomerular Sclerosis ◽  
Tubular Atrophy ◽  
Positive Group ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background and Aims Membranous nephropathy (MN) is the most common cause of biopsied nephrotic syndrome in adults. Recently, it has been reported that the pathogenesis of MN may be associated with an activation of the complement system. The pathway of activation is not clearly established. The intensity of C3 deposition could be a good marker of this activation in MN as has been shown in other diseases (IgA nephropathy, crescentic GN). The aim of this study is to evaluate clinical-pathological data in a cohort of patients with MN and the significance of glomerular C3 staining as a possible predictor of renal outcomes. Method We analysed patients with idiopathic MN biopsied in our department between January 2000 and December 2019, excluding those who had no material for IF (n = 115). The patients were divided into positive (87 cases) and negative (28 cases) based on glomerular C3 deposition. We assessed the clinical and histological characteristics and the percentage of spontaneous remission (SR) and end-stage renal disease (ESRD). Results A total of 115 patients with MN were followed with a median follow-up of 65 (25-161) months. We found no differences in baseline characteristics between both groups, with the exception that patients with C3 deposit had less albumin at the time of biopsy that negative patients [2.4 (2-2.9) vs 2.8 (2.3-3.1) g/dl, P=0.011)]. Patients with C3-negative had a higher percentage of SR than patients with C3-positive (75 vs 24%, P = 0.000) and less need for immunosuppressive treatment (18 vs 56%, P =0.001). At the most recent follow-up, C3-positive group had higher creatinine [1.42 (0.8-1.7) vs 0.97 (0.71-1) mg/dl, P=0.045] and proteinuria [1.64 (0.08-3.2) vs. 0.62 (0.05-0.79) g / 24h, P = 0.039]. Regarding histology, we found no differences in glomerular sclerosis, tubular atrophy and interstitial fibrosis. The renal survival analysis showed no statistically significant differences between both groups (P = 0.091). We analysed a subgroup of patients (n = 23) with antibodies against the phospholipase receptor on blood at the time of the biopsy (13/23 were positive). 84% of this positive group presented C3-positive in the renal biopsy vs 25% of the C3-negative group (P =0.008). Conclusion Patients without C3 staining show a higher rate of SR and less need for immunosuppressive treatment than patients with C3-positive. These results would support the theory that complement activation in this entity can play an important role. It is possible that these patients with negative C3 deposit represent a MN with evolution to SR and in these patients and that these patients do not need immunosuppressive treatment.

scholarly journals Urinary Periostin Excretion Predicts Renal Outcome in IgA Nephropathy

2016 ◽  
Vol 44 (6) ◽  
pp. 481-492 ◽  
Author(s):  
Jin Ho Hwang ◽  
Jung Pyo Lee ◽  
Clara Tammy Kim ◽  
Seung Hee Yang ◽  
Jin Hyuk Kim ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Renal Fibrosis ◽  
Interstitial Fibrosis ◽  
Renal Outcome ◽  
Glomerular Sclerosis ◽  
Enzyme Linked Immunosorbent Assay ◽  
Matricellular Protein ◽  
Tubular Atrophy ◽  
Renal Outcomes ◽  
Group 3

Background: Periostin is a matricellular protein and plays a vital role in tissue regeneration, fibrosis and wound healing. However, data about its significance in nephrology are limited. We investigated the correlation between urinary periostin excretion and its clinical significance including renal histologic findings and prognosis in IgA nephropathy (IgAN). Methods: Of 399 patients from a glomerulonephritis cohort recruited between January 2009 and December 2014, 314 were enrolled. Serum and urine periostin (uPOSTN) were measured using enzyme-linked immunosorbent assay. We divided the patients into 3 groups by uPOSTN/creatinine (uPOSTN/Cr): group 1 (undetectable), group 2 (lower than the median) and group 3 (higher than the median). Results: The uPOSTN level was correlated with pathologic classifications and both initial and final IDMS-MDRD estimated glomerular filtration rates (eGFRs; p < 0.001). Histologically, group 3 patients were correlated with severe interstitial fibrosis/tubular atrophy (p = 0.004), interstitial inflammation (p = 0.007), hyaline arteriolosclerosis (p = 0.001) and glomerular sclerosis (p < 0.001). A higher initial uPOSTN/Cr level was associated with a greater decline in eGFR during follow-up (p = 0.043 when initial eGFR ≥60; p = 0.025 when eGFR <60 ml/min/1.73 m2), and the renal outcomes with end-stage renal disease (ESRD; p = 0.003), ESRD and/or eGFR decrease of >30% (p = 0.033) and ESRD and/or eGFR decrease of >50% (p = 0.046) occurred significantly more in group 3. In multivariate analysis, uPOSTN group 3 (hazards ratio 2.839, 95% CI 1.013-7.957; p = 0.047) was independently associated with ESRD in IgAN patients. Conclusion: uPOSTN/Cr value at initial diagnosis correlated with renal fibrosis and predicted the renal outcomes in patients with IgAN. It could be a promising urinary biomarker for renal fibrosis.

P0136COMPARISON OF SECONDARY IGA NEPHROPATHY OF ANKYLOSING SPONDYLITIS AND PRIMARY IGA NEPHROPATHY IN CLINICAL AND PATHOLOGICAL FEATURES

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Qin Xue ◽  
Guang Li Zhang ◽  
Zebo Tian
Keyword(s):  
Retrospective Study ◽  
Ankylosing Spondylitis ◽  
Iga Nephropathy ◽  
Interstitial Fibrosis ◽  
Renal Involvement ◽  
Control Group ◽  
Pathological Features ◽  
Tubular Atrophy ◽  
The Difference ◽  
Clinical And Pathological Features

Abstract Background and Aims Renal involvement is one of the most common extra-articular complications caused by ankylosing spondylitis (AS). The main pathological manifestation is secondary IgA nephropathy(SIgAN) in Chinese AS patients. The difference between SIgAN and primary IgAN (PIgAN) remains unclear due to the lack of cases. Therefore, the aim of this retrospective study was to compare the clinical and pathological features of SIgAN of AS (SIgAN-AS) and PIgAN, to detect the pathogenesis of SIgAN Method Clinical characteristics and pathological data were collected in patients who were diagnosed with IgAN by renal biopsy in our hospital from Jan 2008 to Oct 2018. Patients with SIgAN-AS were recruited by the ratio 1:5 of patients with primary IgAN as the control group in the study. Fifteen patients with SIgAN-AS and Seventy-five patients with PIgAN were enrolled in this retrospective study. Results There were 15 cases in AS group, including 13 male and 2 female. The cohort of 75 patients with PIgAN included 34 male and 41 female. There were more males in AS group 13/15 (86.7%) vs 37/75(49.3%) ,P &lt; 0.05. Compared with PIgAN patients, SIgAN-AS patients had higher incidences of hematuria( 13/15(86.7%)vs 44/75 (58.7%) , P &lt; 0.05), lower levels of 24-hour urinary protein(0.85±0.68 vs 1.57±1.54g, P &lt; 0.05), but higher levels of eGFR (CKD-MDRD formula) (117.60±37.33 vs 85.35±31.36, P &lt; 0.05),eGFR (CKD-EPI formula) (128.01±41.58 vs 92.75±36.09, P &lt; 0.05), Albumin (44.67±3.48 vs 41.09±7.07 g/L, P &lt; 0.05) ESR (43.20 ±33.94 vs 18.79±16.26mm/h, P &lt; 0.001) , and CRP (21.19±30.61 vs 2.11±4.58mg/L, P &lt; 0.001) . From the perspective of renal pathology of PIgAN, SIgA-AS patients had fewer incidences of renal tubular atrophy / interstitial fibrosis of nephropathy (P &lt;0.05). The immunohistostaining analysis showed higher incidences of dominant deposits of single IgA in mesangial cell area (P &lt; 0.05). Conclusion Patients with SIgAN-AS is more common in male and display a milder progression than those with primary IgAN. Majority of the SIgAN-AS can be improved with early intervention.

scholarly journals Oxford MEST classification in Iranian patients with IgA nephropathy, with regards to extracapillary proliferation; a single center experience

2020 ◽  
Vol 5 (1) ◽  
pp. e14-e14
Author(s):  
Mohsen Akhavansepahi ◽  
Maryam Rafieyan ◽  
Hamid Nasri
Keyword(s):  
Iga Nephropathy ◽  
Interstitial Fibrosis ◽  
Laboratory Data ◽  
Clinical Findings ◽  
Reference Laboratory ◽  
Tubular Atrophy ◽  
Single Center Experience ◽  
Medical Centers ◽  
Two Samples ◽  
The Mean

Introduction: IgA nephropathy is the most common glomerulonephritis in the world. Objectives: In this study we aimed to find the relationship between morphological lesions of Oxford classification for IgA nephropathy with clinical findings and some laboratory data. Patients and Methods: All kidney biopsy conducted from 2009 to 2019 conducted in medical centers and were sent to a reference laboratory in Isfahan. All kidney biopsies included two samples for immunofluorescence and light microscopy. After definitive diagnosis of IgAN (IgA nephropathy), the slides were examined to classify the disease based on the Oxford-MEST (M; mesangial hypercellularity, E; endocapillary hypercellularity, S; segmental glomerulosclerosis, and T; tubular atrophy/interstitial fibrosis and also C; crescent) classification. Results: Our study on 238 biopsy proven IgA nephropathy patients showed that 78 patients (32.8%) were male. Mean ± SD of age individuals was 38.00 ±13.68 years. The mean± SD of serum creatinine level was 1.42 ± 0.79 mg/dL and the mean ± SD of proteinuria was 1780.94 ± 1168.75 mg/d. Our study showed no significant association between M, E and S variables with patients’ age. However, a statistically significant relationship between T variable and patients’ age (P=0.028) was detected. Furthermore, morphologic variables of M, E and S were not significantly associated with proteinuria while T variable was positively associated with the quantity of proteinuria (P=0.021). Conclusion: The association of tubular atrophy/interstitial fibrosis (T variable) with quantity of proteinuria showed significance of interstitial lesion on the prognosis IgA nephropathy.

scholarly journals The Correlation Analysis between the Oxford Classification of Chinese IgA Nephropathy Children and Renal Outcome -A retrospective cohort study

2019 ◽  
Author(s):  
Heyan Wu ◽  
Zhengkun Xia ◽  
Chunlin Gao ◽  
Pei Zhang ◽  
Xiao Yang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Iga Nephropathy ◽  
Interstitial Fibrosis ◽  
Cohort Analysis ◽  
Immunosuppressive Agents ◽  
Renal Outcome ◽  
Oxford Classification ◽  
Tubular Atrophy ◽  
Independent Risk Factors

Abstract Background The 2016 Oxford Classification's MEST-C scoring system predicts outcomes in adults with IgA nephropathy, but it lacks large cohort validation in children with IgAN in China. We would like to verify that the MEST-C score can be used to predict the renal outcome of children with IgAN.Methods A retrospective cohort analysis of data from 1243 Chinese children with IgAN who underwent renal biopsy in Jinling Hospital from 2000 to 2017 was performed and investigated with glomerular filtration rate (eGFR),24h urine proteinuria(24h-UP)and blood pressure(BP) at biopsy and during follow-up.The renal pathology was based on Oxford Classification of IgAN.Results We confirm that BP was significantly correlated with mesangial hypercellullarity(M), endocapillary hypercellularity(E), tubular atrophy/ interstitial fibrosis(T) and crescents (C) .There was a significant correlation between eGFR and segmental glomerulosclerosis(S), T and C .24h-UP and all pathological indexes were significantly correlated. S and T were shown to be independent risk factors associated with renal outcomes in our group. Kaplan-Meier revealed that M [log-rank, chi-squared(χ2)=14.679, P=0.000 ], S(χ2=31.508,P=0.000),T (χ2=78.893, P=0.000),C(χ2=16.603, P=0.000) were associated with renal outcome.In univariate analyses, M(HR 2.167, 95% CI, 1.445~3.251, P = 0.000),S(HR 3.081, 95% CI, 2.038~4.658, P = 0.000), T(HR 7.911, 95% CI, 4.670~13.400,P = 0.000) and C(HR3.346, 95% CI, 1.818~6.156,P = 0.000) lesions were significant predictors of renal outcome. In a multivariate analysis, only S(HR 2.742, 95% CI, 1.805~4.164,P = 0.000) and T(HR 6.633, 95% CI, 3.897~11.289,P = 0.000)were shown to be independent risk factors .Conclusions We found that S and T lesions were valid in predicting a poor outcome in our group.E, S, T and C lesion were important basis for doctors to choose glucocorticoids and immunosuppressive agents in the treatment of IgAN, while T and C lesion were often the basis for doctors to use RAS blockers cautiously.

Immunoglobulin A-nephropathy in Russian population: clinical and morphological presentation and long-term prognosis

2019 ◽  
Vol 23 (6) ◽  
pp. 45-60
Author(s):  
V. A. Dobronravov ◽  
T. O. Muzhetskaya ◽  
D. I. Lin ◽  
Z. Sh. Kochoyan
Keyword(s):  
Iga Nephropathy ◽  
Cox Regression ◽  
Interstitial Fibrosis ◽  
Immunoglobulin A ◽  
Renin Angiotensin System ◽  
Composite Endpoint ◽  
Russian Population ◽  
Progression Rate ◽  
Tubular Atrophy ◽  
Lower Egfr

 AIM. The analysis of incidence, clinical and morphological manifestations, and the prognosis of IgA nephropathy in the Russian population.PATIENTS AND METHODS. Six hundred cases with primary IgA nephropathy (IgAN) from 1999 to 2019 were enrolled in the single-center retrospective study. Demographic and clinical parameters, morphrology data, and the treatment were analyzed. Three hundred forty seven patients were included in follow-up study. The following outcomes were evaluated: the occurrence of complete (PR) or partial remission (CR), death from all causes, the need for renal replacement therapy (RRT). The composite endpoint (RRT or eGFR decrease ≥ 50 % from the time of biopsy) was used to evaluate the risk of IgAN progression and associated factors.RESULTS. The period-average incidence of IgAN cases was 20.5 % of all indication biopsies and 31.7 % of primary immune glomerulopathies (with gradual increase to 41,5 % in last 5 years). At the time of the kidney biopsy, the proteinuria was 2.20 (1.10; 4.40) g/24h, eGFR – 69 ± 32 ml / min / 1.73 m2. Proportions of cases with arterial hypertension and with eGFR <60 ml / min / 1.73 m2 were 75 % and 36 %, respectively. The prevalence of histological changes in accordance with the MEST-C classification was as follows: M1 – 40.5 %, E1 -22.9 %, S1-70.2 %, T1-22 %, T2 – 9 %, C1-16.7 %, C2 – 4.4 %. Combined deposits of IgA and IgM (71.1 % of cases) were more frequent compared to IgA and IgG (9,6 %). In the followup period (27 (11; 61) month), 6 deaths from all causes were registered (1.7 %). The 10-year cumulative renal survival was 75 % (by dialysis) and 55 % (by composite endpoint). PR registered in 26 % of cases, CR – 24 %. PR / CR was more frequent in patients who received immunosuppression compared with patients on renin-angiotensin system blockers only (60 % vs. 40 %, p = 0.001). In multivariable Cox regression the independent factors associated with the risk of IgAN progression were: male gender, a younger age, higher blood pressure and hematuria, lower eGFR, interstitial fibrosis/ tubular atrophy (≥50 %), peritubular capillaritis and the presence of any crescents. Compared to the cohorts of other ethnic or geographical affiliation, analyzed IgAN cases were found to have more severe clinical and morphological presentations and faster progression rate.CONCLUSION. While being the most common glomerulopathy, IgAN in the Russian population has more pronounced clinical and morphological presentations and an unfavorable prognosis.

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