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Background Data on use of interleukin (IL)-1 blockers in kidney transplant recipients (KTRs) with familial Mediterranean fever (FMF) are very limited. We aimed to evaluate the efficacy and safety of anakinra and canakinumab in the transplantation setting. Methods In this retrospective cohort study, we included KTRs who suffered from AA amyloidosis caused by FMF and treated with anakinra or canakinumab (study group, n = 36). Using propensity score matching, we selected 36 patients without FMF or amyloidosis from our database of 696 KTRs as the control group. Primary outcomes were patient and graft survival. Biopsy-confirmed graft rejection, changes in estimated glomerular filtration rate (eGFR), high-sensitivity CRP (hsCRP), erythrocyte sedimentation rate (ESR), proteinuria and number of monthly attacks were secondary outcomes. Results All KTRs with FMF began IL-1 blocker therapy with anakinra and nine (25%) were switched to canakinumab. Overall death was more frequent in the study group (19.4% vs 0%) (P = .005); however, overall graft loss was comparable between study (27.8%) and control groups (36.1%) (P = .448). Five- and 10-year graft survival rates were significantly higher in the study group (94.4% and 83.3%, respectively) than in the control group (77.8% and 63.9%, respectively) (P = .014 and P Conclusions Anakinra and canakinumab are effective in KTRs suffering from FMF; however, the mortality rate may be of concern.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic led to rapid vaccine development and large global vaccination schemes. However, patients with immune-mediated kidney disease, chronic kidney diseases and kidney transplant recipients show high non-response rates to vaccination despite more than three vaccinations and, consequently, reduced viral clearance capacity when infected while receiving certain immunosuppressants, carrying an elevated risk for coronavirus disease 2019 (COVID-19)-related morbidity and mortality. SARS-CoV-2 evolution has been characterized by the emergence of novel variants and spike mutations contributing to waning efficacy of neutralizing antibodies. To this end, the therapeutic field expands from vaccination towards a combined approach of immunization, pre-exposure prophylaxis and early post-exposure treatment using direct-acting antivirals and neutralizing monoclonal antibodies to treat early in the disease course and avoid hospitalization. This expert opinion paper from the Immunonephrology Working Group of the European Renal Association (ERA-IWG) summarizes available prophylactic and/or early treatment options (i.e. neutralizing monoclonal antibodies and direct-acting antivirals) of SARS-CoV-2-infected patients with immune-mediated kidney disease, chronic kidney disease and kidney transplant recipients.

Background This study aims to examine the association of LIM zinc finger domain containing 1 (LIMS1) genotype with allograft rejection in an independent kidney transplant cohort. Methods We genotyped 841 kidney transplant recipients for the LIMS1 rs893403 variant by Sanger sequencing followed by polymerase chain reaction confirmation of the deletion. Recipients who were homozygous for the LIMS1 rs893403 genotype GG were compared with the AA/AG genotypes. The primary outcome was T cell–mediated or antibody-mediated rejection (TCMR or ABMR, respectively) and secondary outcome was allograft loss. Results After a median follow-up of 11.4 years, the rate of TCMR was higher in recipients with the GG genotype (n = 200) compared with the AA/AG genotypes (n = 641) [25 (12.5%) versus 35 (5.5%); P = 0.001] while ABMR did not differ by genotype [18 (9.0%) versus 62 (9.7%)]. Recipients with the GG genotype had 2.4 times higher risk of TCMR than those who did not have this genotype [adjusted hazard ratio2.43 (95% confidence interval 1.44–4.12); P = 0.001]. A total of 189 (22.5%) recipients lost their allografts during follow-up. Kaplan–Meier estimates of 5-year (94.3% versus 94.4%; P = 0.99) and 10-year graft survival rates (86.9% versus 83.4%; P = 0.31) did not differ significantly in the GG versus AA/AG groups. Conclusions Our study demonstrates that recipient LIMS1 risk genotype is associated with an increased risk of TCMR after kidney transplantation, confirming the role of the LIMS1 locus in allograft rejection. These findings may have clinical implications for the prediction and clinical management of kidney transplant rejection by pretransplant genetic testing of recipients and donors for LIMS1 risk genotype.

BACKGROUND AND AIMS Kidney transplantation (KT) is the preferred treatment option for patients with end-stage renal disease (ESRD) as it provides better patient survival and quality of life. While longer graft survival is maintained with potent immunosuppressive drugs used to prevent allograft rejection, de novo malignancy development after transplantation has become a substantial issue. In this study, we aimed to investigate the incidence and risk factors of post-transplant malignancy in kidney transplant recipients, including its demographic, clinical and laboratory features. METHOD A retrospective cohort study was conducted at two tertiary care kidney transplant centers in the same province. We recruited adult (>18 years of age) kidney transplant recipients who underwent kidney transplantation between 1986 and 2020. We excluded KT recipients who lost to follow-up in the early post-transplant period and/or with incomplete records. Malignancies that occurred after graft failure were also excluded. Kidney transplant recipients with malignancy were matched to KT recipients without malignancy using a 1:1 ratio. RESULTS In this study, 2750 eligible patients were reviewed for the development of malignancy after kidney transplantation. A total of 278 KT recipients (10.1%) had biopsy confirmed malignancies during the follow-up period. The median post-transplant follow-up time was 217 months (IQR: 148–290 months). The most common malignancies were nonmelanoma skin cancer (28.8%), urinary tract cancer (16.5%), Kaposi's sarcoma (9.7%), gastrointestinal tract cancer (6.5%) and post-transplant lymphoproliferative disease (6.5%). The median time from kidney transplantation to the diagnosis of malignancy was 127 months (IQR: 62–198 months). In comparison to the control group, patients with malignancy were older (P ≤ 0.001), had a higher family history of malignancy (P ≤ 0.001), had a greater history of smoking (P = 0.005), and usage of erythropoietin in the pre-transplant period was higher (P ≤ 0.001). There was no significant difference between the two groups in terms of gender and the type of induction therapy. Overall mortality was higher in patients with malignancy (OR: 2.491 [CI: 1.586–3.912], P CONCLUSION The most common malignancy in kidney transplant recipients was found to be non-melanoma skin cancer. Elderly recipients, patients with a family history of malignancy and patients using erythropoietin in the pre-transplant period should be closely monitored for the development of malignancy.

Background and Aims Hematuria is one of the most common laboratory findings in nephrology practice. In different regions of the world, the etiologic causes differ. To date, there is no enough data regarding the clinical and histopathologic characteristics of primary glomerular diseases (PGD) patients with hematuria in our country. Method Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database between May 2009 and June 2019. The data of all PGD patients over the age of 16 years who were diagnosed with renal biopsy and had hematuria data were included in the study. The biopsy samples were processed using a light microscopy and immunofluorescence examination. Demographic characteristics such as age, sex, indications for biopsy, primary glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were also recorded. Hematuria was defined as the presence of at least 5 red blood cells/hpf. Results Data of 3394 patients were included to the study after the exclusion of patients with secondary glomerulonephritis and patients with missing biopsy findings. While 1699 (50.1%) patients had hematuria, 1695 (49.9%) patients did not have hematuria. Demographic, laboratory, and histopathological characteristics of patients with and without hematuria are given in Table. Patients with hematuria had statistically higher systolic blood pressure (SBP), serum blood urea nitrogen, creatinine, albumin, levels and urine pyuria, however, these patients had statistically lower age, body mass index, presence of hypertension and diabetes, eGFR, 24-hour proteinuria, serum total, HDL and LDL-cholesterol and C3 levels when compared with patients without hematuria. Figure depicted the etiologic causes of patients with and without hematuria. According to histopathological findings, number of global sclerotic glomeruli, cellular and fibrocellular crescents, the levels of mesangial proliferation, endocapillary proliferation, exudative changes in glomeruli, severe tubular atrophy, interstitial inflammation, subendothelial deposition, moderate and severe IgA and C3 deposition were found to be significantly higher and the levels of basal membrane thickening, interstitial fibrosis, subepithelial deposition, severe IgG staining were found to be significantly lower in patients with hematuria. Conclusion This is the first multicenter national report regarding the demographic and histopathologic data of PGD patients with or without hematuria. Hematuria, a feature of nephritic syndrome, was found at a higher than expected in the PGDs presenting with nephrotic syndrome in our national database.

Background and Aims According to the data of the Turkish Society of Nephrology-Glomerular Diseases Working Group (TSN-GOLD Working Group), IgA nephropathy is the most common primary glomerular disease in Turkey. The purpose of this study was to investigate the epidemiological and clinical data of IgA nephropathy patients in Turkey. Method 4399 patients with primary glomerular diseases from 47 centers who were followed up between May 2009 and May 2019 were included in the study conducted by TSN-GOLD Working Group. 524 patients were excluded due to lack of pathological data. Among the remaining patients, demographic, clinical and laboratory data of 994 patients with IgA nephropathy were analyzed. Results The median age of the patients was 37 (28-47) years, and 37.3% of them were female. The laboratory and clinical data at the time of diagnosis is presented in Figure-1, and biopsy indications are described in Figure-2. The median number of glomeruli was 16 (IQR: 3.5-4.3), sclerotic glomeruli was 2 (IQR: 1-5), and segmental sclerotic glomeruli was 1 (IQR: 1-2). Exudative changes, subendothelial and subepithelial deposition were present in 566 patients (56.9%), 46 patients (4.6%) and in 38 patients (3.8%), respectively. 662 (66.1%) and 611 of the patients (61.4%) had tubular atrophy and interstitial fibrosis in varying degrees, respectively. 672 (%67.6) and 416 patients (%41.9) had interstitial inflammation and vascular changes, respectively. In immunofluorescence staining, 18%, 30.1%, 4.4%, 68% of the patients had IgG, IgM, C1q and C3 positivity, respectively. Crescentic glomeruli were detected in 227 patients (3.3 ± 3.1 glomeruli). Patients with crescentic glomeruli had significantly higher proteinuria and lower eGFR than the patients without [2203 mg/day (15-26078) vs 1807 mg/day (15-29112); p=0.001; 55.3 ml/min/1.73 m2 (3.72-141.9) vs 72 ml/min/1.73 m2 (3.84-150.81); p Conclusion In this study we found that, the most common presentation of IgA nephropathy patients in our country was asymptomatic urinary abnormalities followed by nephritic and nephrotic syndrome. Higher proteinuria and lower eGFR values in patients with crescentic glomeruli, support the adoption of crescentic lesions in the new Oxford classification (MEST-C) to predict more precise outcome of IgA nephropathy patients. The high number of patients to whom the Oxford classification was applied provided us with the opportunity to examine the clinical reflections of pathological features. Evaluation of the follow-up data of the patients will give us the possibility to reveal the effect of initial clinical and pathological features on clinical findings and renal outcome.

Background and Aims In IgA nephropathy (IgAN), which is characterized by mesangial IgA accumulation, there is the formation of circulating autoantibodies against galactose deficient IgA1s (Gd-IgA1). IgG / Gd-IgA1 immunocomplexes accumulate in the glomerular mesangium and play a role in the pathogenesis of IgAN. Recent studies have suggested a relationship between glomerular IgG deposition and the severity of glomerular inflammation. However, detection of the presence and severity of IgG in routine immunofluorescence microscopy (IFM) may fail. This study aims to investigate whether IgG positivity detected by IFM is associated with poor renal prognostic indicators and whether renal prognosis can be predicted according to IgG positivity. Method 4399 patients who were enrolled between May 2009-June 2019 in database of Turkish Society of Nephrology, Glomerular Diseases Working Group (TSN-GOLD) including 44 centers were evaluated. After exclusion criteria, 994 primary IgAN patients were included in the study. Glomerular IgG negative and positive patients were compared by means of Oxford classification scores, histopathological evaluations, proteinuria, creatinine, albumin, blood pressures. IgG positive patients were divided into subgroups according to the grade of the IFM positivity. The relationship between IgG positivity and poor prognosis criteria were evaluated. Results Demographic and biochemical findings of glomerular IgG positive and negative patients at the time of biopsy are shown in Table 1. No difference was found between the groups. There was no difference in the demographic and biochemical findings at the time of biopsy in IgG subgroup analyses (Table 2). There was no difference between the histopathological and Oxford MEST scores of the subgroups. Glomerular IgG positivity was not associated with diastolic blood pressure, systolic blood pressure, urea, uric acid, age, eGFR, albumin, proteinuria (p> 0.05 for all, r= -0.084, r= -0.102, r= -0.006, r=0.062, r= 0.014, r= -0.044, r= -0.061, r= -0.066, r= 0.150, respectively). Conclusion Glomerular IgG positivity detected by routine IFM in IgAN is not associated with poor renal prognostic indicators. It is difficult to predict renal prognosis by looking at the severity of IgG positivity at the baseline evaluation.

Background and Aims The largest data on the epidemiology of primary glomerular diseases (PGD) are obtained from the databases of countries or centers. Here, we presented the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD)Working Group. Method The data of patients who underwent renal biopsy and diagnosed as primary glomerular disease were recorded in the database prepared for the study. Between May 2009 and May 2019, a total of 4399 patients from 47 centers were evaluated. Basal data of 3875 patients were analyzed after exclusion of those lacking light microscopy and immunofluorescence findings. Results The mean age was 41.5 ± 14.9 years. Of the patients, 1690 were female (43.6%) and 2180 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGH was IgA nephropathy (25.7%), followed by membranous nephropathy (25.6%) and FSGS (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. Mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. Median proteinuria was 3300 (IQR: 1467-6307) mg / day, mean serum creatinine, estimated GFR and albumin values were 1.4 ± 1.5 mg / dl, 80.7 ± 39.1 ml / min and 3.2 ± 0.9 g / dl, respectively. Conclusion In Turkey, the incidence of IgA nephropathy patients have become more common than membranous nephropathy among PGD patients diagnosed with renal biopsy.

Background. Patients on kidney replacement therapy comprise a vulnerable population and may be at increased risk of death from coronavirus disease 2019 (COVID-19). Currently, only limited data are available on outcomes in this patient population. Methods. We set up the ERACODA (European Renal Association COVID-19 Database) database, which is specifically designed to prospectively collect detailed data on kidney transplant and dialysis patients with COVID-19. For this analysis, patients were included who presented between 1 February and 1 May 2020 and had complete information available on the primary outcome parameter, 28-day mortality. Results. Of the 1073 patients enrolled, 305 (28%) were kidney transplant and 768 (72%) dialysis patients with a mean age of 60 ± 13 and 67 ± 14 years, respectively. The 28-day probability of death was 21.3% [95% confidence interval (95% CI) 14.3–30.2%] in kidney transplant and 25.0% (95% CI 20.2–30.0%) in dialysis patients. Mortality was primarily associated with advanced age in kidney transplant patients, and with age and frailty in dialysis patients. After adjusting for sex, age and frailty, in-hospital mortality did not significantly differ between transplant and dialysis patients [hazard ratio (HR) 0.81, 95% CI 0.59–1.10, P = 0.18]. In the subset of dialysis patients who were a candidate for transplantation (n = 148), 8 patients died within 28 days, as compared with 7 deaths in 23 patients who underwent a kidney transplantation Conclusions. The 28-day case-fatality rate is high in patients on kidney replacement therapy with COVID-19 and is primarily driven by the risk factors age and frailty. Furthermore, in the first year after kidney transplantation, patients may be at increased risk of COVID-19-related mortality as compared with dialysis patients on the waiting list for transplantation. This information is important in guiding clinical decision-making, and for informing the public and healthcare authorities on the COVID-19-related mortality risk in kidney transplant and dialysis patients.

Background and Aims Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in the adult population. The aim of our study is to determine the demographic and clinical characteristics and biopsy findings of the patients diagnosed with MN in our country. Method The study included 991 patients with renal biopsy who were followed in 47 nephrology clinics and enrolled in the database of Turkish Nephrology Primary Glomerular Diseases (TSN-GOLD) Working Group between May 1, 2009, and June 1, 2019. The demographic and clinical characteristics and biopsy findings of all patients included in the study were analyzed. Results The mean age of patients included in our study was 46±14.8 years and the gender distribution was (M / F, 582/409). 80.3% (n = 796) of the patients had nephrotic proteinuria at the time of diagnosis. Before the biopsy, the PLAR2 antibody was examined in 137 patients and positive in 71.5% (n = 98) patients. Hypertension was present in 34.6% (n = 343) of the patients. Mean estimated glomerular filtration rate was 93.9±32.8 ml/min/1.73 m2, albumin level was 2.7 ± 0.2 mg/dL, proteinuria level in urine was 7,1±6,4 gr/day and total cholesterol level was 295±3.7 mg/dL. On light microscopy, baseline membrane thickening was found in 92% (n=907) and interstitial inflammation in 53% (n=524) specimens. In immunofluorescence staining, 3 (+) IgG accumulation was highest in 578 (58.3%) of the specimen. Conclusion This multicentre study is the largest study to analyze clinical, laboratory, and biopsy specimens of MN patients at the time of biopsy in our country. The findings are consistent with the literature. Prospective follow-up results of these patients will help us to understand the renal prognosis and treatment response better.

Background and Aims Several glomerular disease registries in Europe, and they shared their data; however, there was a lack of current data on trends of primary glomerulopathy in Turkey. Glomerular disease patterns can change geographical areas and populations decade by decade. Our aim is to present Turkey's primary glomerular disease pattern from 2009 to 2017. Method 3878 native kidney biopsy records were assessed in the Turkish Society of Nephrology Glomerulopathy (TSN-GOLD) Working Group Registry. Secondary disease (lupus, etc.) and transplant biopsies do not enroll in the registry. These records divided into four periods, before 2009, 2009 to 2013, 2013 to 2017, and 2017 to current. Results A total of 3858 patients (M = 2173, K = 1685) were examined. There was no difference in the distribution of number of patients according to periods (Q1 = 968, Q2 = 960, Q3 = 968, Q4 = 962). Nephrotic syndrome was the most common biopsy indication in all quarters (58.6%, 53%, 44.1%, 51.6%, respectively). Glomerulopathy types; Membranous GN (29.9%, n = 290) was the first in Q1 and IgA nephropathy (17.7%, n = 172) was the second. IgA nephropathy (28.5%, n = 274) was the most common glomerulopathy in Q2, while Membranous GN (25.5%, n = 245) was the second most common. IgA nephropathy (29.9%, n = 285) was the most common glomerulopathy in Q3, followed by FSGS (22.9%, n = 222) and Membranous GN. In Q4, FSGS (26.8, n = 258) was the most common glomerulopathy, followed by IgA nephropathy (26.7%, n = 257) and Membranous GN (24.5%, n = 236) (Figure 1). Conclusion While biopsy indications did not change over the years, the incidence of FSGS and IgA nephropathy gradually increased.

Background and Aims The combination of new onset haematuria, proteinuria and declining GFR, often referred to as the nephritic syndrome, constitute a strong indication for renal biopsy. Several different disease entities can underlie these findings, many of them requiring prompt treatment. Epidemiological studies of glomerular diseases are in general hampered by the strong influence of differences in biopsy practices. However, there is reason to believe that such differences are less pronounced in cases of acute/subacute nephritic syndrome. The aim of the present study is to compare the frequencies of diseases causing the nephritic syndrome in Japan and Europe using data from renal biopsy registries. Method Data were retrieved from national or regional renal biopsy registries in Japan, Sweden, Norway, Scotland, Austria, Croatia and Turkey from 2007-01-01 to 2018-12-31. Only patients biopsied due to acute or subacute nephritic syndrome, defined as a combination of new onset haematuria, sub-nephrotic proteinuria and reduced GFR, were included. Demographic, clinical and laboratory data were collected from the time of renal biopsy. Results A total number of 2789 patients from Japan and 1202 patients from Europe were included. Among the Japanese patients, 46% were women, median age was 67 years (IQR 54-74) and median creatinine concentration at the time of renal biopsy was 198 µmol/L (IQR 109-343). In Europe, 43% were women, median age was 58 years (IQR 42-70) and median creatinine concentration was 210 µmol/L (IQR 127-390). Figures from the different European countries are presented in Table 1. The most common diagnosis in both Japan and Europe was ANCA-associated nephritis (44% in Japan, 34% in Europe), followed by IgA nephritis (8.5% in Japan, 18.6% in Europe) and acute tubulointerstitial nephritis (6.2% in Japan and 7.1% in Europe). Conclusion We describe preliminary data from a large multicenter cohort of patients presenting with the nephritic syndrome. This clinical presentation is most often encountered in the 6th and 7th decade of life and the distribution between men and women is fairly equal. The most common underlying cause of the nephritic syndrome in both Japan and Europe is ANCA-associated nephritis.

Background and Aims Membranous nephropathy (MN) is the most common cause of glomerulonephritis in non-diabetic adults. B-cell dysfunction is an important pathway involved in pathogenesis. We, therefore, investigated effects of rituximab (RTX) on the outcome of patients with relapsed or refractory primary MN. Method In this retrospective analysis, 48 patients with primary MN were evaluated. Patients, who had relapsed or refractory biopsy-proven disease, with a nephrotic-range proteinuria despite at least six months of prior immunosuppressive therapy using corticosteroids, calcineurin inhibitors or mycophenolic acid derivatives, which were used according to KDIGO guidelines. All patients received at least 2 doses of 375 mg/m2 of RTX. Proteinuria and serum albumin levels of the patients were recorded at baseline, and 3,6,12,18 and 24 months. Results Of 48 patients who participated in this study, 27(56.3%) were male. Mean age was 45.3±17.2 years. Results of serum anti-phospholipase-A2-receptor (PLA2R) were available in 31 patients, 23 of whom (74%) were positive. Proteinuria levels at 3, 6, 12, 18, and 24 months after treatment with RTX showed a significant decrease when compared to baseline (p Conclusion Treatment with RTXiseffective in patients suffering from relapsed or refractory primary MN.

Background and Aims Allograft rejection following BK virus nephropathy (BKVN) is an important cause of allograft loss in kidney transplant recipients. However, the effect of rejection type on allograft survival in patients with BKVN has not been described previously. This study aimed to investigate the relationship between allograft rejection type and graft survival in patients with BKVN. Method We retrospectively analyzed the data of 159 kidney transplant recipients diagnosed BKVN and followed-up in our center between January 2009 and December 2019. BKVN was diagnosed by persistent viremia of more than 10000 copies/mL for four weeks or allograft biopsy. Vascular, obstructive or other non-parenchymal etiologies for allograft dysfunction were excluded. All patients were investigated for the presence of anti-HLA antibody at 6 and 12 months after BKVN diagnosis. Luminex solid-phase assay was used to investigate Class I and Class II PRA and MFI values greater than 1000 were accepted as positive. Allograft biopsy was performed in patients with progressive graft dysfunction or the presence of donor-specific antibodies (DSA) and analyzed according to the Banff Classification. The primary outcomes were defined as allograft loss or the allograft dysfunction which defined as was doubling serum creatinine levels. Results Patients were followed-up median 70 (IQR 13-198) months after kidney transplantation. Demographic data and clinical characteristics are provided in the table. 28 kidney transplant recipients suffered from allograft rejection after BKVN. Median rejection time to rejection after BKVN was 9 (IQR 5-164) months. 3 patients (18,8%) in the AMR group and 1 patient (8,3%) in the TCMR group experienced graft loss during follow-up. The mean serum creatinine levels at the last clinical visit were significantly higher in the AMR group compared to the TCMR group (1,9±0,8 vs 1,3±1,2 mg/dl; p=0,002). In multivariate analysis, AMR was an independent risk factor for allograft dysfunction (HR, 1,735; 95% CI 1,060 to 2,839; p=0,028). Conclusion The occurrence of AMR after BKVN is an important indicator of allograft dysfunction compared to TCMR. DSA screening should be routinely used in this group for early diagnosis and treatment of AMR.

Background and Aims Rapidly progressive glomerulonephritis (RPGN) is a clinical condition that develops due to different etiologic causes, characterized by a rapid and progressive decrease in renal function and progresses to end-stage renal failure in weeks to months if not treated. In our study, diagnostic and demographic characteristics of patients diagnosed with RPGN by biopsy, clinical and laboratory findings in our country were investigated. Method Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group database between May 2009 and June 2019. Demographic characteristics such as age, sex, indications for biopsy, diagnosis of the glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were recorded. The data presented is cross-sectional and includes application data for the biopsy period. According to their types, RPGN patients were classified as type 1 (anti-GBM related), type 2 (immunocomplex related) and type 3 (immune-negative; “pauci-immune”). Results After exclusion of 46 patients with missing data, 200 patients (mean age 47.9 ± 16.7 years, 44% female) were included in the study which constitutes 5.2% of the total glomerulonephritis database (total number of patients: 3875). Hypertension was present in 62 patients (31.0%) and diabetes was present in 18 patients (9.0%). Renal biopsy was performed in 147 (73.5%) patients due to nephritic syndrome (RPGN included). 80.2% of the patients' biopsies were performed in nephrology clinics and 19.8% of them were performed in radiology clinics. ANCA positivity was found in 121 (60.5%) patients (proteinase 3-ANCA was positive in 55 and myeloperoxidase-ANCA positive in 66 patients). Type 1 RPGN was detected in 11 (5.5%), type 2 RPGN in 42 (21%) and type 3 RPGN in 147 (73.5%) patients. In 21 patients (10.5%), biopsy revealed RPGN with advanced chronic changes (fibrous global sclerotic glomeruli, advanced interstitial fibrosis and tubular atrophy). Mean serum creatinine was 4.2 ± 3.4 mg/dl, median glomerular filtration rate was 18 (10-37) ml/min and proteinuria 2100 (1229-3526) mg/day according to CKD-EPI formula. The mean number of glomeruli in the biopsies was 18.8 ± 10.6 and the number of crescentic glomeruli was 9.9 ± 7.7 (ratio: 52.7%) (Figure). The patients were divided into 3 groups according to their crescentic glomeruli ratios. The proportion of crescentic glomeruli is 10-50% in group 1, 50-80% in group 2, and >80% in group 3. The demographic, laboratory and histopathological characteristics of the groups are given in Figure. It was observed that urea and creatinine increased and calcium and hemoglobin decreased with increasing crescentic glomerular ratio. Conclusion Our study provides valuable demographic, clinical, laboratory and histopathological data about RPGN in our country. Our data are generally compatible with the literature. In our study, advanced chronic histopathological findings were prominent in the biopsy of 21 patients. Early biopsy should be performed to confirm the diagnosis of RPGN and to avoid unnecessary intensive immunosuppressive therapy. In addition to the treatments applied, detailed data, including patient and renal survival are needed.

Background and Aims Fabry Disease (FD) is a lysosomal glycosphingolipid storage disorder. Kidneys are affected in FD. Despite enzyme replacement theraphy (ERT), some patient still have an ongoing deterioration of renal functions probably secondary to inappropriate immune responses. There is no data regarding NK cell subtypes and the exact relation with B-T lymphocytes and NK cells in patients with FD. We aimed to analyze subtypes of NK cells in patients with FD and compared these results with healthy subjects. Additionally, we analyzed the renal biopsy (RBx) materials of our patients prior to ERT in terms of B, T and NK cell infiltration and compared these cells in some of our FD patients after 4 years of ERT. Method 15 patients with FD and 10 healthy controls were included in the study. Of 15 patients, 8 patients were receiving agalsidase alfa or beta. Blood samples obtained from both group were taken into 2 ml EDTA tube to evaluate peripheral NK cell subgroups according to CD56 and CD16 expression and CD3, CD4, CD8 expression to determine subtypes T cells. These cells were evaluated by flow cytometry technique. The RBx of 10 patients with FD were evaluated before ERT and 3 were reevaluated after 4 years of ERT in terms of T-B, NK and plasma cells infiltration of kidney. The paraffin blocks of specimens were examined with HE, PAS, Masson-trichrome and toluidine blue staining and frozen blocks were examined with red-oil 10 staining Results The characteristics of patients with FD and healthy controls are depicted in Figure 1. Except proteinuria, there were no significant differences in terms of biochemical parameters between two groups. According to flow-cytometric analyses, total percentage of NK and T helper 1, 2, 17 and Treg cells of FD patients are similar to controls (Fig 1). When we analyzed subgroups of NK cells and determined that CD56dimCD16dimNK cells were increased, however, CD56dimCD16brightNK cells were decreased in patients with FD compared to controls (Fig1). We performed RBx of 10 patients. The percentages of CD3, 4, 8 positive Tcells, CD19, CD20 positive B cells, CD 16, 56 positive NK cells and CD 138 positive plasma cell infiltration are shown in Fig 2. According to RBx findings prior to ERT, all patients had interstistial fibrosis (IF), podocyte vacuoles (PV), podocyte inclusion (PI), CD3, CD4, CD16, CD56 positivity at different levels. In contrast, almost none of the patients had CD19, CD20, CD138 positivity. We rebiopsied 3 patients after 4 years of ERT and reevaluated specimens in terms of IF, global and segmental sclerosis (SS), PV, PI and the percentages of CD3, CD4, CD16, CD56, CD 19, CD 20, CD 138 positivity. 2 rebiopsied patients from the same family had increased serum creatinine and proteinuria despite 4 years of regular ERT. In the second biopsy of these two patients, we found that global sclerosis (GS), IF and PI are increased and abundant CD19, CD20 and CD 138 positive cells were seen which were almost absent in the first biopsy. The other patient from another family, had mildly increased proteinuria and had no creatinine increment after 4 years of ERT. In this patient, we found that GS, SS, IF, PI and arterial sclerosis were increased, however CD 3, 4, 8 positive T cells were decreased and no changes in the percentage of CD16, 56 positive NK cells. We could not demonstrate CD 19, 20, 138 positivity in the latter patient. Conclusion Our group, for the first time, demonstrated that CD56dimCD16dimsubtype of NK cells is increased in FD which is closely associated with cellular cytotoxity in normal population. Additionaly, CD 19 and CD 20 positive B cell infiltration are increased and glomerular and interstitial changes are advanced in some patients with ongoing kidney function deterioration despite 4 years of regular ERT. Hence, infiltration of Bcells in the kidneys of patients under ERT might change our therapeutic approaches in the near future. Further studies are needed to clarify the exact roles of B T and NK cells in FD.

Background and Aims Although hematuria is the cardinal symptom of IgA nephropathy (IgAN), its effects on the outcome have not been studied extensively. We, therefore, aimed to analyze the association between microhematuria and clinicopathological features as well as outcome parameters in adult patients with IgAN. Method 129 adults with IgAN, diagnosed by kidney biopsy, and followed up for a median duration of 54.5 (IQR: 24.25-92.75) months, were included in this retrospective study. Urinary sediment analyses during the bouts of macrohematuria were not taken into consideration. For the purpose of this analysis, microhematuria was described as ≥5 red blood cells per high-power field (RBCs/hpf) and classified as mild (5-9 RBCs/hpf), moderate (10-19 RBCs/hpf), or severe (≥20 RBCs/hpf). Study outcome (event) was defined as at least a 50% reduction in baseline eGFR or development of stage 5 chronic kidney disease (eGFR Results Demographic, clinical, laboratory and histopathological features of patients at the time of diagnosis are summarized in the table. Usage of ACEi/ARBs [75/81 (92.5%) vs 45/48 (93.75%), p=0.803], fish oil [30/81 (37%) vs 19/48 (39.5%), p=0.773], azathioprine [16/81 (19.7%) vs 10/48 (20.8%), p=0.882] and mycophenolic acid derivatives [14/81 (17.2%) vs 11/48 (22.9%), p=0.434] were comparable among the patients with and without microhematuria. Corticosteroids were more frequently used in patients with microhematuria [41/81 (50.6%) vs 17/48 (35.4%)], although this difference was not statistically significant (p=0.093). Overall 30 patients (23.2%) reached the study outcome, and there were no differences between patients with (19, 23.4%) and without (11, 22.9%) microhematuria (p=0.944). Kaplan-Meier analysis revealed that event free survival rates were similar across study groups: 77.1% for patients without microhematuria; while 80% for mild, 77.3% for moderate, and 72.7% for severe microhematuria (p=0.436) (Figure). Microhematuria did not predict the study outcome when multivariable Cox regression analyses were performed [HR: 1.847 (95% CI: 0.696-4.904), p=0.218]. Throughout the follow-up, microhematuria disappeared (dropped below 5 RBCs/hpf) in 43 patients (53%), 8 of whom (18.6%) reached the study outcome as compared to 11 patients (28.9%) with persistent microhematuria (p=0.273). Disappearance of microhematuria was not a predictor of study outcome, as well [HR: 0.386 (95% CI: 0.068-2.180), p=0.281]. Conclusion Microhematuria is not associated with renal outcomes of adult patients with IgAN.

Background and Aims Therapeutic plasma exchange (PEX) has an increasingly long list of indications in recent years, in addition to immunosuppressive therapies in many life-threatening immune activations, it provides significant improvements in mortality and morbidity. In this study, we aimed to investigate the laboratory and clinical effects of PEX performed with nephrologic indication in our clinic. Method The records of 67 patients (36 females, 31 males; mean age, 45.7±15.8 years) who underwent PEX with nephrological indication between 2012 and 2017 in our clinic were retrospectively reviewed. Characteristics of the patients such as, indications of PEX, laboratory values and number of PEX sessions were recorded. In addition, clinical responses were also evaluated. Results When a total 398 PEX sessions were examined, the most common indication (40.3%) was acute humoral rejection after renal transplantation, followed by granulomatosis with polyangiitis (19.4%) and thrombotic microangiopathy (9%), respectively. The average of the PEX sessions was 5.94. There was a statistically significant increase in the bicarbonate value when the arterial blood gas of the patients was evaluated after PEX (p=0.002). However, no significant difference was observed in the pH and ionized calcium values (p=0.135, p=0.969, respectively) (Table 1). When all the patients were evaluated, there was no significant change in hemoglobin values (p=0.174), but platelet values decreased significantly (p=0.011) after PEX. However, when the patient group admitted with thrombotic microangiopathy was examined, it was observed that platelet count increased and LDH level decreased significantly (p=0.063, p=0.028, respectively). When the serum creatinine values of all patients were evaluated, a significant decrease was observed in the serum creatinine values after PEX (p=0.001). In addition, after 2 years following PEX treatment, 70.1% of patients were still alive and 12.5% of patients undergoing PEX for acute humoral rejection were undergoing on hemodialysis treatment. Conclusion According to the results of our study, patients should be followed up especially for acid-base and electrolyte changes after PEX. In addition, therapeutic PEX might be effective in terms of improving morbidity and 2 year-mortality of these patients.