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Background: C4d deposits are present in a substantial proportion of patients with IgA nephropathy (IgAN), indicating the activation of the lectin pathway (LP) of the complement system. It seems that patients with activated LP have worse renal prognosis. The aim of this study was to investigate the prevalence and prognostic significance of C4d in our cohort of patients with primary IgA nephropathy (pIgAN). Methods: Patients with pIgAN were recruited from a hospital register of kidney biopsies of the Department of Nephrology and Dialysis, Dubrava University Hospital, Zagreb. Additional immunohistochemistry staining for C4d was performed on paraffin-embedded kidney tissue, and patients were stratified into being C4d positive or C4d negative. The clinical and histologic features of patients were analyzed and compared regarding C4d positivity. The primary outcome was defined as kidney failure (KF), and predictor variables of KF and renal survival were analyzed. Results: Of a total of 95 patients with pIgAN included in the study, C4d was present in 43 (45.3%). C4d-positive patients had a higher value of systolic (p = 0.039) and diastolic (p = 0.006) blood pressure at diagnosis as well as higher 24 h proteinuria (p = 0.018), serum urate (p = 0.033), and lower eGFR (p < 0.001). C4d-positive patients had worse renal survival (p < 0.001), higher rates of disease progression to KF (p < 0.001), and higher proteinuria (p < 0.001) and lower eGFR (p < 0.001) at the last follow-up. Glomerular C4d was an independent predictor of disease progression to KF (HR = 5.87 [0.95 CI 1.06–32.44], p = 0.032). Conclusions: C4d is an independent predictor of disease progression in patients with pIgAN. C4d may be used as an additional marker of progressive disease course in IgAN. The therapeutic implications of C4d status in IgAN, particularly in terms of complement inhibitors application, are not yet known. [ABSTRACT FROM AUTHOR]

The management strategy for IgA nephropathy (IgAN), has undergone constant improvements since the disease entity was first described 50 years ago. However, it is still unknown how these changes affected the long-term renal survival of IgAN patients. We systematically evaluate changes in IgAN renal survival by searching PubMed, Embase, and the Cochrane Library Database of Systematic Reviews from inception to 19 May 2024. We included a large sample of 103076 IgAN cases from 158 studies. Renal survival rates were 94.16% (95% CI: 94.02% to 94.31%), 88.68% (95% CI: 88.48% to 88.87%), and 78.13% (95% CI: 77.82% to 78.43%) at three, five, and ten-year, respectively. Over the past few decades, there haven’t been any sound changes in the 3-year and 5-year renal survival rates. The kidney survival rate in developed countries is higher than in developing countries. Researchers consistently show that while proteinuria < 1.0 g/24 h, renal survival rates increase dramatically. In IgAN, long-term renal survival fluctuated rather than continuously improving over time. Our system review’s findings indicate that supportive care—the most important recommendation for managing IgAN has shown promising results. The long-term outcomes of IgAN could be significantly improved by the latest developed treatment options.

Objects: We aim to explore the correlation between active/chronic tubulointerstitial injury and renal survival, and to compare their predictive value in patients with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (AAGN). Method: A total of 225 patients with MPO-AAGN diagnosed between February 2004 and December 2020 were included. Survival and univariate/multivariate Cox regression analyses were used to analyze the prognostic value of interstitial inflammation and interstitial fibrosis/tubular atrophy (IF/TA). Results: Of the 225 patients, 73 (32.4%) patients developed end-stage renal disease (ESRD) requiring maintenance dialysis. Interstitial inflammation>50% and IF/TA>50% were important predictors for ESRD in MPO-AAGN in multivariate Cox regression analysis adjusted by age, gender, estimated glomerular filtration rate (eGFR)≤15 ml/min/1.73m2, and normal glomeruli% (classified by <25%, 25–50%, >50%). Furthermore, we conducted stratified Cox regression analysis and found different results in the subgroups of eGFR>15 ml/min/1.73m2 and eGFR≤15 ml/min/1.73m2. Interstitial inflammation>50% and IF/TA>50% were significant risk factors for ESRD in the subgroup of eGFR>15 ml/min/1.73m2, but not or less significant in the subgroup of eGFR≤15 ml/min/1.73m2. Similarly, the survival analysis according to interstitial inflammation>50%/≤50% and IF/TA>50%/≤50% showed significant differences in the subgroup of eGFR>15 ml/min/1.73m2, but not or less significant in the subgroup of eGFR≤15 ml/min/1.73m2. Conclusions: Interstitial inflammation>50% and IF/TA>50% were prognostic factors for renal survival in MPO-AAGN. In particular, interstitial inflammation and IF/TA had a better predictive ability in the subgroup of eGFR>15 ml/min/1.73m2. Key Points • Interstitial inflammation>50% and IF/TA>50% can help to predict renal survival in MPO-AAGN. • Both interstitial inflammation and IF/TA had a better predictive ability in the subgroup of eGFR>15 ml/min/1.73m2than those in the subgroup of eGFR≤15 ml/min/1.73m2. [ABSTRACT FROM AUTHOR]

Abstract Background Hypertensive emergency is a critical disease that causes multifaceted sequelae, including end-stage kidney disease and cardiovascular disease. Although the renin–angiotensin–aldosterone (RAA) system is enormously activated in this disease, there are few reports that attempt to characterize the effect of early use of RAA inhibitors (RASi) on the temporal course of kidney function. Methods This retrospective cohort study was conducted to clarify whether the early use of RASi during hospitalization offered more favorable benefits on short-term renal function and long-term renal outcomes in patients with hypertensive emergencies. We enrolled a total of 49 patients who visited our medical center with acute severe hypertension and multiple organ dysfunction between April 2012 and August 2020. Upon admission, the patients were treated with intravenous followed by oral antihypertensive drugs, including RASi and Ca channel blockers (CCB). Kidney function as well as other laboratory and clinical parameters were compared between RASi-treated and CCB- treated group over 2 years. Results Antihypertensive treatment effectively reduced blood pressure from 222 ± 28/142 ± 21 to 141 ± 18/87 ± 14 mmHg at 2 weeks and eGFR was gradually restored from 33.2 ± 23.3 to 40.4 ± 22.5 mL/min/1.73m2 at 1 year. The renal effect of antihypertensive drugs was particularly conspicuous when RASi was started in combination with other conventional antihypertensive drugs at the early period of hospitalization (2nd day [IQR: 1–5.5]) and even in patients with moderately to severely diminished eGFR (

Resumen: La poliquistosis renal autosómica dominante (PQRAD) es una de las principales causas de insuficiencia renal terminal. Hoy día el conocimiento de sus bases genéticas está permitiendo desarrollar estrategias que eviten la transmisión de la enfermedad. Objetivos: El objetivo del estudio fue analizar la historia natural de la PQRAD en la provincia de Córdoba y diseñar una base de datos que permita agrupar a las familias y a las diferentes mutaciones. Pacientes y métodos: Se incluyen todos los pacientes (n = 678) diagnosticados de PQRAD seguidos en el servicio de Nefrología de Córdoba. Se analizaron de manera retrospectiva diversas variables clínicas (edad y sexo), necesidad de terapia renal sustitutiva (TRS) y genéticas. Resultados: La prevalencia fue de 61 casos por 100.000 habitantes. La mediana de supervivencia renal fue significativamente peor en PKD1 (57,5 años) que en PKD2 (70 años) (Log-rank p = 0,000). Tenemos identificada genéticamente al 43,8% de la población, detectando mutaciones en PKD1 en el 61,2% y en PKD2 en el 37,4% de los casos. La mutación más frecuente fue detectada en PKD2 (c.2159del) en 68 pacientes pertenecientes a 10 familias diferentes. La de peor pronóstico renal fue una mutación truncante detectada en PKD1 (c.9893G>A). Conclusiones: La supervivencia renal de la PQRAD en la provincia de Córdoba es similar a la descrita en la literatura. Con nuestra metodología y estudiando genéticamente al 43,8% de la población, detectamos mutaciones en PKD2 en una mayor proporción de pacientes, en el 37,4% de los casos. Esta estrategia permite conocer las bases genéticas de gran parte de nuestra población con ahorro de recursos. Esto es fundamental para poder ofrecer prevención primaria de la PQRAD mediante diagnóstico genético preimplantacional. Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is one of the main causes of end-stage renal disease. Today, the knowledge of its genetic base has made it possible to develop strategies that prevent the transmission of the disease. Objectives: The objective of the study was to analyze the natural history of ADPKD in the Province of Córdoba and to design a database that allows families and different mutations to be grouped. Patients and methods: All patients (n = 678) diagnosed with ADPKD followed up in the Cordoba nephrology service are included. Various clinical variables (age and sex), genetic variables (mutation in PKD1, PKD2) and the need for renal replacement therapy (RRT) were retrospectively analyzed. Results: The prevalence was 61 cases per 100,000 inhabitants. Median renal survival was significantly worse in PKD1 (57.5 years) than in PKD2 (70 years) (Log-rank p = 0.000). We have genetically identified 43.8% of the population, detecting mutations in PKD1 in 61.2% and in PKD2 in 37.4% of cases. The most frequent mutation was detected in PKD2 (c.2159del) in 68 patients belonging to 10 different families. The one with the worst renal prognosis was detected in PKD1 (c.9893G>A). These patients required RRT at a median age of 38.7 years. Conclusions: The renal survival of ADPKD in the Province of Córdoba is similar to that described in the literature. Mutations in PKD2 were detected in 37.4%. Our strategy allows us to know the genetic bases of our population with a great saving of resources. This is essential to be able to offer primary prevention of ADPKD through preimplantation genetic diagnosis.

Objective To explore the clinicopathological features of anti-glomerular basement membrane (anti-GBM) glomerulonephritis (anti-GBM-GN) and the prognostic values of clinical and laboratory indicators at diagnosis on renal and patient survival.Methods A total of 76 patients (34 males and 42 females) with anti-GBM-GN who were hospitalized in the First Affiliated Hospital of Nanjing Medical University between January 2010 and June 2021 were included in this study. The baseline clinical features, histopathological data from renal biopsies, and predictors of renal and patient survival were retrospectively analyzed.Results Among the 76 patients, the median serum creatinine at diagnosis was 618.0 (350.98, 888.25) μmol/L and the median estimated glomerular filtration rate (eGFR) was 6.62 (4.39, 14.41) mL/min. Of these 76 patients, 55 (72.4%) received initial kidney replacement therapy (KRT) and 39 (51.3%) received plasma exchange or double-filtered plasmapheresis (DFPP). During a median follow-up duration of 28.5 (6.0, 71.8) months, 53 (69.7%) patients progressed to kidney failure with replacement therapy (KFRT) and received maintenance dialysis. Initial KRT (HR = 3.48, 95% CI = 1.22–9.97, p = 0.020) was a significant risk factor for renal survival. During the follow-up, 49 (64.5%) of 76 patients survived. Age (≥60 years, HR = 4.13, 95% CI = 1.65–10.38, p = 0.003) and initial KRT (HR = 2.87, 95% CI = 1.01–8.14, p = 0.047) were predictive of patient survival.Conclusions Among patients with anti-GBM-GN, initial KRT at presentation was predictive of KFRT while older age and initial KRT were associated with higher all-cause mortality.

Few studies tackled the long-term effect of pregnancy on lupus nephritis (LNs); thus, the study aimed to explore the long-term impact of pregnancy on renal outcomes in Egyptian patients with LN. Group I patients included females who had their first pregnancy after LN onset with ≥5 years elapsing after delivery; group II patients included females who had never got pregnant for ≥7 years after LN onset. Data were retrospectively collected at baseline (T0) and the last visit (Tlast). The study included 43 patients in group I and 39 patients in group II. The comparisons between the two groups regarding the characteristics at Tlast showed no significant difference regarding the serum creatinine, estimated glomerular filtration rate (eGFR), renal component of SLICC/ACR Damage Index (SDI) as well as the rate of renal flares, new-onset chronic kidney disease (CKD), progressed CKD and end-stage renal disease. Multivariate regression analysis revealed that systemic hypertension and renal flares were predictors of new-onset/progressed CKD (p = 0.019, OR [95% CI] = 4 [1.3–13]; and 0.022, 13.8 [1.5–128.8], respectively) while pregnancy was not (p = 0.363). Paired comparisons between T0 and Tlast characteristics within each group revealed significant increment of serum creatinine, renal SDI and CKD prevalence; as well as decrement of eGFR in group I (p = 0.004, <0.001, 0.001 and <0.001, respectively) and group II (p = 0.006, <0.001, 0.004 and 0.002, respectively). In conclusion, pregnancy, per se, does not affect the long-term renal outcome in LN patients; however, it is rather dependent on the existence of baseline renal damage and the development of renal flares. [ABSTRACT FROM AUTHOR]

There is a consensus that maintenance therapy should be used to prevent relapse of myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (MPO-AAV), but there is a debate about the optimal duration of maintenance therapy. Therefore, the purpose of this study was to determine whether discontinuation of maintenance therapy in MPO-AAV patients who were in long-term stable remission affects relapse, renal survival and patient survival. Seventy-nine patients with MPO-AAV diagnosed at Xiangya hospital from June 2010 to June 2019 who were in stable remission for at least 18 months following maintenance therapy were included. Patient records were retrospectively reviewed, and based on whether patients discontinued maintenance therapy 18 months after commencing maintenance therapy, patients were assigned into either the withdrawal group (n = 26) or maintenance group (n = 53). The endpoint was the percentage of relapse, relapse-free survival, renal survival and patient survival during follow-up. Ten relapses (38.5%) occurred in the withdrawal group (n = 26) and 8 relapses (15.1%) occurred in the maintenance group (n = 53) (p = 0.020). Compared to the withdrawal group, the maintenance group had similar relapse-free survival (log-rank test p = 0.099). But maintenance group had a better renal survival (p = 0.035), with no difference in patient survival or adverse events. This study suggests that discontinuing maintenance therapy at 18 months following induction of sustained remission leads to a significant increase in the percentage of relapse, and decreases renal survival in patients with MPO-AAV, but does not decrease relapse-free survival or patient survival. [ABSTRACT FROM AUTHOR]

Aim. To analyze the outcomes of C3-glomerulopathy (C3-GP) and determine the associated factors. Materials and methods. A retrospective single-center study included 60 patients with newly diagnosed C3-GP (with primary C3-GP pC3-GP 82%). Of these, 48 (80%) patients had clinical data to assess the following disease outcomes: development of remission and disease progression (by a composite endpoint that included initiation of chronic dialysis or a decrease in estimated glomerular filtration rate eGFR 15 mL/min/1.73 m2 or a decrease in eGFR30% of baseline at the time of renal biopsy). The median follow-up period was 25 (7; 52) months. Results. At early follow-up (median 4 [3; 9] months) remission was registered in 35% of patients; at the end of follow-up, in 48% (for pC3-GP, 32 and 41%). Disease progression occurred in 17 patients. In the overall group the likelihood of achieving early remission was higher with treatment (Exp=6.4, 95% confidence interval CI 1.429.3; p=0.017). Early remission was associated with the presence of remission at the end of follow-up (Exp=6.3, 95% CI 2.218.4; p=0.001). Specific treatment (Exp=0.308, 95% CI 0.1080.881; p=0.028) and late remission (Exp=0.079, 95% CI 0.0170.368; p=0.001) were associated with reduced risk of disease progression in multivariable models (adjusted for eGFR, mean blood pressure). The same results were obtained for the group of patients with pC3-GP. Conclusion. C3-GP is a variant of severe complement-mediated glomerular damage with unfavorable renal prognosis, which requires timely personalized expert-level diagnostics with clarification of etiopathogenesis of the disease followed by therapy aimed at achieving remission to improve outcomes.

Background: Hypertensive emergency is a critical disease that causes multifaceted sequelae, including end-stage kidney disease and cardiovascular disease. Although the renin–angiotensin–aldosterone (RAA) system is enormously activated in this disease, there are few reports that attempt to characterize the effect of early use of RAA inhibitors (RASi) on the temporal course of kidney function. Methods: This retrospective cohort study was conducted to clarify whether the early use of RASi during hospitalization offered more favorable benefits on short-term renal function and long-term renal outcomes in patients with hypertensive emergencies. We enrolled a total of 49 patients who visited our medical center with acute severe hypertension and multiple organ dysfunction between April 2012 and August 2020. Upon admission, the patients were treated with intravenous followed by oral antihypertensive drugs, including RASi and Ca channel blockers (CCB). Kidney function as well as other laboratory and clinical parameters were compared between RASi-treated and CCB- treated group over 2 years. Results: Antihypertensive treatment effectively reduced blood pressure from 222 ± 28/142 ± 21 to 141 ± 18/87 ± 14 mmHg at 2 weeks and eGFR was gradually restored from 33.2 ± 23.3 to 40.4 ± 22.5 mL/min/1.73m2 at 1 year. The renal effect of antihypertensive drugs was particularly conspicuous when RASi was started in combination with other conventional antihypertensive drugs at the early period of hospitalization (2nd day [IQR: 1–5.5]) and even in patients with moderately to severely diminished eGFR (< 30 mL/min/1.73 m2) on admission. In contrast, CCB modestly restored eGFR during the observation period. Furthermore, renal survival probabilities were progressively deteriorated in patients who had manifested reduced eGFR (< 15 mL/min/1.73 m2) or massive proteinuria (urine protein/creatinine ≥ 3.5 g/gCr) on admission. Early use of RASi was associated with a favorable 2-year renal survival probability (0.90 [95%CI: 0.77–1.0] vs. 0.63 [95%CI: 0.34–0.92] for RASi (+) and RASi (-), respectively, p = 0.036) whereas no apparent difference in renal survival was noted for CCB. Conclusions: Early use of RASi contributes to the renal functional recovery from acute reduction in eGFR among patients with hypertensive emergencies. Furthermore, RASi offers more favorable effect on 2-year renal survival, compared with CCB. [ABSTRACT FROM AUTHOR]

Introduction: The data of acute kidney injury (AKI), that is, community-acquired AKI (CA-AKI) and hospital-acquired AKI (HA-AKI) among non- COVID patients from intensive care units (ICU) during the coronavirus disease-2019 (COVID-19) pandemic are scarce. We planned to study the change in the profile of such patients compared to the pre-pandemic era. Materials and methods: This prospective observational study was conducted at four ICUs dealing with non-COVID patients at a government hospital in North India, and was aimed at assessing outcomes, and mortality predictors of AKI among non-COVID patients during the COVID-19 pandemic. Renal and patient survival at ICU transfer-out and hospital discharge, ICU and hospital stay duration, mortality predictors, and dialysis requirement at discharge were evaluated. The current or previous COVID-19 infection, previous AKI or chronic kidney disease (CKD), organ donors, and organ transplant patients were excluded. Results: Among the 200 non-COVID-19 AKI patients, diabetes mellitus (DM), primary hypertension, and cardiovascular diseases were the predominant comorbidities in descending order. The commonest cause of AKI was severe sepsis, followed by systemic infections and post-surgery patients. Dialysis requirements at ICU admission during ICU stay and above 30 days were seen in 20.5, 47.5, and 6.5% of patients, respectively. Incidence of CA-AKI and HA-AKI was 1.24:1, whereas dialysis requirement above 30 days was 0.85:1, respectively. The 30-day mortality was 42%. Hepatic dysfunction [hazard ratio (HR): 3.471], septicemia (HR: 3.342), age above 60 years (HR: 4.000), higher sequential organ failure assessment (SOFA) score (HR: 1.107; p = 0.001), anemia (p = 0.003), and low serum iron (p = 0.001) were important mortality predictors in AKI. Conclusion: Compared to the pre-COVID era, CA-AKI was more common than HA-AKI due to restricted elective surgeries during the COVID-19 pandemic. Acute kidney injury with multiorgan involvement and hepatic dysfunction, elderly age with higher SOFA score and sepsis were predictors of adverse renal and patient outcomes. [ABSTRACT FROM AUTHOR]

Background Acute kidney injury (AKI) is one of the most common complications after cardiac surgery with cardiopulmonary bypass (CPB). Renal transplant recipients (RTRs) have a higher risk of cardiac surgery–associated AKI (CSA-AKI). A relationship has been strongly suggested between AKI and poor long-term graft survival. The main objective was to evaluate the impact of on-pump cardiac surgery on the 1-year renal allograft survival rate. Methods The study population consisted of 37 RTRs and 56 non-RTRs who underwent cardiac surgery between 1 January 2010 and 31 December 2019. They were matched according to age, sex, preoperative glomerular function, diabetes and type of surgery. The primary composite outcome was renal survival, defined as patient survival without the requirement for permanent dialysis or new kidney transplantation at 1 year after surgery. Results The renal survival rate was significantly lower in the RTR group than in the non-RTR group [81% versus 96%; odds ratio 0.16 (95% confidence interval 0.03–0.82), P  = .03]. The proportion of patients who returned to permanent dialysis was higher in the RTR group than in the non-RTR group (12% versus 0%; P  = .02). The proportion of patients with severe AKI was also higher in the RTR group. At 1 year after surgery, serum creatinine level, glomerular filtration rate and all-cause mortality rates were comparable between both groups. Conclusion Patients with a functional renal allograft have a low 1-year renal allograft survival rate after cardiac surgery with CPB. In addition, these patients have significant risks of AKI and acute kidney disease after open-heart surgery. [ABSTRACT FROM AUTHOR]

Introduction: A previous genome-wide association study has identified CARD9 (caspase recruitment domain family member 9) as a susceptibility gene for immunoglobulin A nephropathy (IgAN), which encodes an adapter protein and is related to mucosal immunity. This study aimed to investigate the association of CARD9 variants with the clinicopathological phenotypes and prognosis of IgAN. Methods: Eight single nucleotide polymorphisms within CARD9 were genotyped using Sequenom MassARRAY iPLEX for 986 IgAN patients in this study. Logistic and linear regression analyses adjusted for age and gender were performed to evaluate the effects of CARD9 gene polymorphisms on clinicopathological phenotypes. The Kaplan-Meier method and Cox proportional hazard models were applied to analyze the associations between genetic variants and renal survival. Results: The T allele of rs10747047 was strongly associated with higher levels of serum creatinine (p = 0.005) and lower levels of estimated glomerular filtration rate (p = 0.005). The rs10870149-G and rs10870077-C alleles were associated with elevated 24-h urine protein excretion (p = 0.041 and 0.022, respectively) and more serious segmental glomerulosclerosis lesions (p = 0.005 and 0.041, respectively) in IgAN patients. Carriers with the T allele of rs10781533 and the C allele of rs3812552 also presented with severe segmental glomerulosclerosis lesions (p = 0.001 and 0.010, respectively). Additionally, rs10747047-C and rs10870077-C alleles were independently related to the poor prognosis of IgAN patients after adjustments for covariates (TT vs. CC hazard ratio [HR] = 0.138, 95% confidence interval [95% CI] = 0.022–0.871, p = 0.035; GG vs. CC HR = 0.321, 95% CI = 0.123, 0.836, p = 0.020, respectively). Conclusion:CARD9 variants are associated with disease severity and rapid disease progression for IgAN in a Chinese Han population. [ABSTRACT FROM AUTHOR]

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To explore the clinicopathological features of anti-glomerular basement membrane (anti-GBM) glomerulonephritis (anti-GBM-GN) and the prognostic values of clinical and laboratory indicators at diagnosis on renal and patient survival. A total of 76 patients (34 males and 42 females) with anti-GBM-GN who were hospitalized in the First Affiliated Hospital of Nanjing Medical University between January 2010 and June 2021 were included in this study. The baseline clinical features, histopathological data from renal biopsies, and predictors of renal and patient survival were retrospectively analyzed. Among the 76 patients, the median serum creatinine at diagnosis was 618.0 (350.98, 888.25) μmol/L and the median estimated glomerular filtration rate (eGFR) was 6.62 (4.39, 14.41) mL/min. Of these 76 patients, 55 (72.4%) received initial kidney replacement therapy (KRT) and 39 (51.3%) received plasma exchange or double-filtered plasmapheresis (DFPP). During a median follow-up duration of 28.5 (6.0, 71.8) months, 53 (69.7%) patients progressed to kidney failure with replacement therapy (KFRT) and received maintenance dialysis. Initial KRT (HR = 3.48, 95% CI = 1.22–9.97, p = 0.020) was a significant risk factor for renal survival. During the follow-up, 49 (64.5%) of 76 patients survived. Age (≥60 years, HR = 4.13, 95% CI = 1.65–10.38, p = 0.003) and initial KRT (HR = 2.87, 95% CI = 1.01–8.14, p = 0.047) were predictive of patient survival. Among patients with anti-GBM-GN, initial KRT at presentation was predictive of KFRT while older age and initial KRT were associated with higher all-cause mortality. [ABSTRACT FROM AUTHOR]

Background. The efficacy and indications of tonsillectomy in IgA nephropathy (IgAN) remain uncertain. Methods. We performed a retrospective cohort study of 452 patients with primary IgAN, including 226 patients who received tonsillectomy and 226 controls selected by propensity score matching who had never undergone tonsillectomy. Study outcomes were clinical remission defined as negative hematuria and proteinuria on three consecutive visits over a 6-month period, the endpoint defined as end-stage renal disease or an irreversible 100% increase in serum creatinine from the baseline value. In addition, we further analyzed the critical level of proteinuria in the efficacy of tonsillectomy and the correlation between MEST-C score and tonsillectomy. Results. Up to December 2019, the follow-up period lasted 46 ± 23 months (12-106 months). Kaplan-Meier and multivariate Cox regression analysis revealed that tonsillectomy was beneficial for clinical remission and renal survival. Whether proteinuria was ≥ 1 g/24h or >1 g/24h, the clinical remission and renal survival rates were greater in patients treated with tonsillectomy than without. When the pathological damage was mild or relatively severe, tonsillectomy may be beneficial to clinical remission or renal survival. Conclusions. Tonsillectomy had a favorable effect on clinical remission and delayed renal deterioration in IgAN. In addition to patients with early stage IgAN, it may also be beneficial to IgAN patients with higher levels of proteinuria and relatively severe pathological damage. [ABSTRACT FROM AUTHOR]

Although patients with light chain amyloidosis (AL) may present with codeposition of amyloid and immune complexes (ICs) in renal biopsies, data on clinical characteristics and prognostic value of renal IC deposition are limited. A total of 73 patients with AL amyloidosis who were newly diagnosed by renal biopsy in Xijing Hospital (Xi'an, China) were divided into two groups (IC and non-IC groups). As a result, renal IC deposition was found in 26% of patients. Patients with IC deposition were associated with more urinary protein excretion and lower serum albumin. Notably, patients in the non-IC group achieved higher hematological overall response rate (81.5% vs. 47.4%, p = 0.007) and =VGPR rate (75.9% vs. 39.8%, p = 0.004) compared with those in IC group. Renal response rate was also higher in the non-IC group (63% vs. 31.6%, p = 0.031). With the median follow-up time of 19 months, a significantly worse overall survival was observed in patients with the IC group as compared with those without renal IC deposition in the Kaplan-Meier analysis (p = 0.036). Further multivariate analysis demonstrated that renal immune complex deposition was associated with worse overall survival in patients with AL amyloidosis (HR 5.927, 95% CI 2.148-16.356, p = 0.001). [ABSTRACT FROM AUTHOR]

The management strategy for IgA nephropathy (IgAN), has undergone constant improvements since the disease entity was first described 50 years ago. However, it is still unknown how these changes affected the long-term renal survival of IgAN patients. We systematically evaluate changes in IgAN renal survival by searching PubMed, Embase, and the Cochrane Library Database of Systematic Reviews from inception to 19 May 2024. We included a large sample of 103076 IgAN cases from 158 studies. Renal survival rates were 94.16% (95% CI: 94.02% to 94.31%), 88.68% (95% CI: 88.48% to 88.87%), and 78.13% (95% CI: 77.82% to 78.43%) at three, five, and ten-year, respectively. Over the past few decades, there haven't been any sound changes in the 3-year and 5-year renal survival rates. The kidney survival rate in developed countries is higher than in developing countries. Researchers consistently show that while proteinuria < 1.0 g/24 h, renal survival rates increase dramatically. In IgAN, long-term renal survival fluctuated rather than continuously improving over time. Our system review's findings indicate that supportive care-the most important recommendation for managing IgAN has shown promising results. The long-term outcomes of IgAN could be significantly improved by the latest developed treatment options.

BackgroundSeveral lines of evidence implicate that there are distinct differences between patients with myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (GBM) antibody double-seropositive patients (DPPs) and single-positive patients. Hence, we conducted a retrospective study from a single center in China to analyze the clinical and pathological features, and prognosis of DPPs.Methods109 patients with MPO-ANCA-associated vasculitis (MPO-AAV), 20 DPPs and 23 patients diagnosed with anti-GBM disease from a large center in China were included in this study. The ratio of patients with renal biopsy in three groups were 100%, 50% and 100%, respectively. Their clinical and pathological characteristics, and outcomes were analyzed. The intensity of immune deposits in the kidney at diagnosis was detected by immunofluorescence (IF). Furthermore, multivariate Cox hazard model analysis was used to assess the clinical and histological predictors of end-stage renal disease (ESRD) and death for DPPs.ResultsIn our study, we found that patients in the DPPs group were older than the other two groups (p = 0.007, MPO-AAV vs. DPPs; p < 0.001, DPPs vs. anti-GBM). The DPPs group had a higher value of serum creatinine (p = 0.041) and lower estimated glomerular filtration rate (eGFR) (p = 0.032) compared with MPO-AAV patients. On the contrary, the DPPs group had a lower serum creatinine (p = 0.003) compared with patients with anti-GBM group. The proportion of patients with cardiac system involvement in the DPPs group was higher than anti-GBM patients (p = 0.014). Cellular crescents could be generally observed in renal biopsy of DPPs and patients with anti-GBM glomerulonephritis. In addition, Bowman’s capsule rupture was more common in DPPs than MPO-AAV patients (p = 0.001). MPO-AAV had a better renal and overall survival outcome than DPPs (p < 0.001). There was no significant difference of renal and overall survival outcome between DPPs and patients with anti-GBM disease. The incidence of ESRD in DPPs was negatively associated with lymphocyte count (HR 0.153, 95% CI 0.027 to 0.872, p = 0.034) and eGFR (HR 0.847, 95% CI 0.726 to 0.989, p = 0.036). Elevated serum creatinine was confirmed as a risk factor of both renal (HR 1.003, 95% CI 1.000 to 1.005, p = 0.019) and patient survival in DPPs (HR1.461, 95% CI 1.050 to 2.033, p = 0.024).ConclusionIn summary, compared with anti-GBM disease, DPPs tended to involve multi-organ damage rather than limited to the kidney. It is highlighted that serologic DPPs have a worse renal and patient prognosis than MPO-AAV. Moreover, we found that the risk factors of renal survival of DPPs include low lymphocyte count, elevated serum creatinine and reduced eGFR, and serum creatinine can predict patient survival.

Introduction: IgA nephropathy (IgAN) is one of the most common forms of glomerulonephritis worldwide. It leads to end-stage renal disease (ESRD) in many patients. At the time of diagnosis, risk stratification is of paramount importance in planning proper management in individual cases. Several studies have been conducted to determine the utility of various demographic, clinical, laboratory, and pathological features on renal biopsy to stratify the risk of disease progression and predict the likely outcome. This review summarizes the emerging data on demographic, clinical, laboratory, and histological prognosis along with risk factors associated with renal outcomes in patients with IgAN. Methods: For this review, we searched DOAJ (Directory of Open Access Journals), PubMed/Medline, Web of Science, Scopus, Embase, and Google Scholar, using keywords including; "IgA nephropathy," "IgA nephritis," "IgAN," "Berger's disease," "Berger's syndrome," "chronic glomerulonephritis, "prognostic factors," "risk factors," "risk stratification," "renal survival," "ESRD," "MEST classification," "MEST-C classification" and "Oxford Classification." To identify other relevant studies, we manually scanned the bibliographic lists of the identified studies and reviewed articles from January 2009 through December 2020. All relevant articles were carefully reviewed, and relevant information was extracted for this narrative review. Results: A total of 152 articles were retrieved from the above literature database searches. The abstracts were carefully reviewed to identify 35 articles containing information on prognostic factors and longterm renal survival in IgAN patients. Relevant information was collected and summarized for this review. The main focus was on using demographic, clinical, and laboratory features, especially serial changes in these parameters during follow-up, for this purpose. Recently a standardized, evidencebased formulation has been devised to evaluate and categorize pathological features on renal biopsy to augment and refine the risk stratification and prognostic value of traditional risk factors; it is popularly known as the Oxford classification of IgAN. There have been numerous validation studies in various ethnic groups that have proven its clinical utility. Conclusion: In conclusion, the clinicians should also take into account the pathologic variables according to the revised Oxford classification in addition to demographic, clinical, and laboratory parameters for early and reliable risk stratification and prognostication in individual patients at the time of diagnosis in IgAN for optimal management and ultimate improvement in long-term outcomes. [ABSTRACT FROM AUTHOR]

Aim: Study the long-term outcome of the patients with LN and identify the baseline factors that can predict the long-term outcome of these patients Methods: All biopsy-proven LN patients who attended our regular 'lupus nephritis' clinic from 2013 to 2021 were studied. Data of these patients were collected from the hospital patient records. Standard therapy was given as per the KDIGO guidelines, and the renal response was evaluated according to KDIGO outcome criteria. Cox' regression analysis was used to determine predictors of chronic kidney disease (persistent doubling of serum creatinine with creatinine ≥1.5 mg%). Kaplan–Meier analysis was used for renal survival. Results: Eighty patients with at least 1 year of follow-up were included. Median age of onset was 24 years (IQR18-35). Median follow up was 6.5 years (IQR 3–10). World Health Organisation renal biopsy profile was Class I 1(1.2 %), Class II 6(7.5 %), Class III 9(11.2 %), Class IV 36(45 %), Class V 18(22.5 %) and Mixed Class IV + V 10 (12%). Complete remission was achieved in 63.75%, 70 % and 66.6% patients at 1, 2 and 5 years, respectively. Survival with normal renal function was 88.5 %, 85.8% and 60 % at 5, 10 and 15 years, respectively. Risk factors for poor outcome on univariate analysis were presence of Raynaud's phenomena-hazard ratio(HR) 7.78 (CI 1.944–31.207; p <.004), baseline hypertension-HR 5.356 (CI 1.479–19.403; p <.011), tubulointerstitial involvement-HR 1.076 (CI 1.032–1.222; p <.001), time to complete response-HR 1.036 (CI 1.036–1.067; p <.02), serum creatinine at 6 months HR 10.51 (CI 2.19–50.39; p <.003), failure to achieve complete response at 2 years HR 6.271 (CI 1.567–25.092; p <.009) and the number of nephritic flares HR 1.868(CI 1.103–3.164 ; p <.02). Renal relapses were quite common, with 1.8 flares per 10 patient-years of follow up. Infection was the most common cause of death, with bacterial lower respiratory infections and pulmonary tuberculosis being the most common. Conclusions: Apart from conventional risk factors, other predictive factors like the presence of Raynaud's phenomenon, tubulointerstitial fibrosis and tubular atrophy on kidney biopsy, and initial response to induction therapy by 6 months have a significant impact on the long-term outcome in patients with LN. [ABSTRACT FROM AUTHOR]

Background: The triglyceride–glucose (TyG) index is a simple, novel and reliable surrogate marker of insulin resistance. However, evidence for the prognostic impact of an elevated TyG index on IgA nephropathy (IgAN) is limited. Therefore, we evaluated the relationship between the TyG index and the risk of renal progression in IgAN. Method: This cohort study involved biopsy-proven IgAN between January 2009 and December 2018 in West China Hospital, in which patients were assigned to two groups based on the cut-off value of TyG using receiver operating characteristic (ROC) curves. A 1:1 matched-pair analysis was established to optimize the bias in IgAN by propensity score matching (PSM). The TyG index was calculated as ln [fasting triglyceride (mg/dL) × fasting glucose (mg/dL)/2]. The composite endpoint was defined by eGFR decreased ≥50% of the baseline level, end-stage kidney disease (ESKD), renal transplantation and/or death. Univariable and multivariable Cox proportional hazard models were applied to confirm the predictive value of the optimal marker. Results: Before PSM, a total of 1210 participants were ultimately included. During a median follow-up period of 55.8 months (range 37.20–79.09 months), 129 participants progressed to the composite endpoint (10.7%). After PSM, 366 patients were enrolled in the matched cohort, of whom 34 (9.3%) patients reached the endpoints. Based on the cut-off value of the TyG index, patients were divided into the low TyG index group (TyG ≤ 8.72, n = 690) and the high TyG index group (TyG > 8.72, n = 520). Further analysis demonstrated that a higher TyG index was significantly associated with a higher risk of reaching composite endpoints in IgAN patients in both the unmatched and matched cohorts (before PSM: HR 2.509, 95% CI 1.396–4.511, p = 0.002; after PSM: HR 2.654, 95% CI 1.299–5.423, p = 0.007). Conclusion: A high TyG index is associated with a higher risk of renal progression. [ABSTRACT FROM AUTHOR]

Objective: To define predictors of response, time to response, flares, and long-term renal outcome in an inception cohort of proliferative lupus nephritis (PLN). Methods: We included 100 patients (80% female; mean age 31 ± 13 years) with biopsy-proven PLN (III, IV, III/IV + V). Clinical, laboratory, histological and therapeutical parameters were recorded at baseline, 6, 9, 12, 18, 24, 36, 72 months, time of flare, and last follow-up visit. Logistic and Cox-regression models were applied. Results: After induction treatment (69% received cyclophosphamide (CYC) and 27% mycophenolic acid (MPA)), partial (PR) or complete (CR) response was achieved in 59% (26% CR, 33% PR) and 67% (43% CR, 24% PR) of patients at 3 and 6 months, respectively; median time to PR was 3 months (IQR 5) and median time to CR was 6 months (IQR 9). Baseline proteinuria <1.5 g/day correlated with a shorter time to CR (HR 1.77) and with CR at 3, 6, and 9 months (OR 9.4, OR 5.3 and OR 3.7, respectively). During 100-month median follow-up, 33% of patients had ≥1 renal flares (median time: 38 months). Proteinuria >0.8 g/day at 12 months was associated with a higher risk of flares (OR 4.12), while MPA and mixed classes with lower risk (OR 0.14 and OR 0.13, respectively). Baseline proteinuria >2 g/day and 12-month proteinuria >0.8 g/day correlated with a shorter time to flare (HR 2.56 and HR 2.57, respectively). At the end of follow-up, 10% developed stage 3–4 chronic kidney disease (CKD), and 12% end-stage renal disease (ESRD). Twelve-month proteinuria >0.8 g/day (OR 10.8) and interstitial fibrosis/tubular atrophy >25% (OR 7.7) predicted CKD or ESRD at last visit. Conclusions: Baseline proteinuria <1.5 g/day predicted time to CR. Twelve-month proteinuria >0.8 g/day correlated with flares (ever) and time to flare and, along with baseline interstitial fibrosis/tubular atrophy >25%, predicted CKD or ESRD at the last visit. [ABSTRACT FROM AUTHOR]

Background: Immunoglobulin A nephropathy (IgAN) is the most common type of primary glomerulonephritis. Since most patients have a relatively benign renal prognosis, long-term follow-up is required. During such a long course of disease, relapse of IgAN is occasionally observed after upper respiratory tract infection or without any trigger. However, little is known about the impact of relapse on long-term renal outcomes. Methods: In this retrospective cohort study of biopsy-proven primary IgAN, we analyzed the association of 5-year therapeutic responsiveness (relapse) with the subsequent development of end-stage kidney disease (ESKD) using a 5-year landmark analysis (Cox model) and explored predictors of relapse from histological and clinical data at baseline. Results: Among 563 patients from the exploratory cohort, most relapses (13.7%) occurred within 5 years after treatment. Using 5-year landmark analysis, among 470 patients, 79 developed ESKD during a median follow-up period of 155 months. Even after adjustment for clinicopathological relevant confounders, hazard ratios (95% confidence intervals) in the relapse and non-responder groups compared with the remission group were 2.86 (1.41–5.79) and 2.74 (1.48–5.11), respectively. Among 250 patients who achieved remission within 5 years, proteinuria, eGFR, mesangial hypercellularity, endocapillary hypercellularity, segmental sclerosis, and crescent, but not interstitial fibrosis/tubular atrophy, were independent predictors of 5-year relapse in multivariable logistic regression analysis, Conclusions: Both relapsers and non-responders had similarly strong association with ESKD in patients with IgAN. We also confirmed the predictors of relapse 5 years after renal biopsy, which may guide the treatment strategies for patients with IgAN who occasionally relapse after remission. [ABSTRACT FROM AUTHOR]

Introduction: IgA nephropathy (IgAN) is one of the most common forms of glomerulonephritis worldwide. It leads to end-stage renal disease (ESRD) in many patients. At the time of diagnosis, risk stratification is of paramount importance in planning proper management in individual cases. Several studies have been conducted to determine the utility of various demographic, clinical, laboratory, and pathological features on renal biopsy to stratify the risk of disease progression and predict the likely outcome. This review summarizes the emerging data on demographic, clinical, laboratory, and histological prognosis along with risk factors associated with renal outcomes in patients with IgAN. Methods: For this review, we searched DOAJ (Directory of Open Access Journals), PubMed/Medline, Web of Science, Scopus, Embase, and Google Scholar, using keywords including; "IgA nephropathy," "IgA nephritis," "IgAN," "Berger’s disease," "Berger’s syndrome," "chronic glomerulonephritis, "prognostic factors," "risk factors," "risk stratification," "renal survival," "ESRD," "MEST classification," "MEST-C classification" and "Oxford Classification." To identify other relevant studies, we manually scanned the bibliographic lists of the identified studies and reviewed articles from January 2009 through December 2020. All relevant articles were carefully reviewed, and relevant information was extracted for this narrative review. Results: A total of 152 articles were retrieved from the above literature database searches. The abstracts were carefully reviewed to identify 35 articles containing information on prognostic factors and long-term renal survival in IgAN patients. Relevant information was collected and summarized for this review. The main focus was on using demographic, clinical, and laboratory features, especially serial changes in these parameters during follow-up, for this purpose. Recently a standardized, evidence-based formulation has been devised to evaluate and categorize pathological features on renal biopsy to augment and refine the risk stratification and prognostic value of traditional risk factors; it is popularly known as the Oxford classification of IgAN. There have been numerous validation studies in various ethnic groups that have proven its clinical utility. Conclusion: In conclusion, the clinicians should also take into account the pathologic variables according to the revised Oxford classification in addition to demographic, clinical, and laboratory parameters for early and reliable risk stratification and prognostication in individual patients at the time of diagnosis in IgAN for optimal management and ultimate improvement in long-term outcomes.

IntroductionIgA nephropathy (IgAN) encompasses a wide range of clinical and histology features. Some patients present without hematuria, with or without hypertension, still rapidly progress in renal function. Renal pathology of this part of patients were predominant intrarenal arteriolar lesions, rarely presented glomerular proliferative lesions. We aim to investigate the clinical and pathological characteristics and prognosis of these IgAN patients and initially explore whether the abnormal activation of complement is involved in the intrarenal arteriolar lesions of IgAN.MethodsA total of 866 patients with renal biopsy-proven IgAN diagnosed at Beijing Anzhen Hospital were recruited. IgAN patients without intrarenal arteriolar lesions and proliferative lesions were excluded (n = 115), the rest were divided into arteriolar lesions group (n = 202) and proliferative lesions group (n = 549). Among them, 255 patients were regularly followed up for at least 1 year. Renal biopsy tissues of 104 IgAN patients were stained for complement components by immunohistochemistry and immunofluorescence.ResultsCompared with proliferative lesions group, the arteriolar lesions group experienced high percentage of hypertension (p = 0.004), low percentage of gross hematuria (p = 0.001), microscopic hematuria (p < 0.001) and less initial proteinuria (p = 0.033). Renal survival between the two groups was not significantly different (p = 0.133). MBL, C4d, FH and FHR5, C3c, and MAC deposited on intrarenal arteriole in arteriolar lesions group. Compare with the proliferative lesion group, the arteriolar lesions group exhibited a higher intensity of C3c deposition on the intrarenal arterioles (p = 0.048). C3c and CD31 co-deposited on intrarenal arterioles area in patients with intrarenal arteriolar lesions.ConclusionRenal survival of the IgAN patients in arteriolar lesions group was not better than those in proliferative lesions group. Abnormal activation of complement may be involved in the pathogenesis of arteriolar damage through the injury of endothelial cells in this clinical phenotype of IgAN.

Abstract Background Whether cigarette smoking is associated with the progression of immunoglobulin A nephropathy (IgAN) remains uncertain; therefore, we aimed to evaluate the effect of cigarette smoking on the prognosis of IgAN. Methods We divided 1239 IgAN patients from West China Hospital of Sichuan University who met the inclusion criteria into smoker (current or former) and non-smoker groups. The endpoint was end-stage renal disease (ESRD: eGFR 50%. Kaplan–Meier, correlation, logistic regression and Cox proportional hazards analyses were performed. The association between cigarette smoking and IgAN was further verified by propensity-score-matched cohort analysis. Results During the mean follow-up period of 61 months, 19% (40/209) of the smoker group and 11% (110/1030) of the non-smoker group reached the study endpoint (p

• A total of 386 patients were enrolled the study. The cohort has a relatively large sample size. • Renal pathological data were also included in the prognostic analysis of double-seropositive patients (DPPs). • The related predictors of renal outcome and patient outcome were all studied. • The results of this study have guiding significance for the treatment of anti-GBM-GN, AAV and DPPs in the future. To explore the clinical characteristics of double-seropositive patients (DPPs) with anti-glomerular basement membrane (Anti-GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA). We collected patients with both ANCA and anti-GBM positive glomerulonephritis who were hospitalized in the Department of Nephrology at the First Affiliated Hospital of Nanjing Medical University from January 2010 to August 2022. Retrospective analysis of the baseline clinical characteristics of patients and follow-up to explore relevant factors affecting renal and patient survival. A total of 386 patients, including 69 ANCA negative anti-GBM glomerulonephritis patients, 296 anti-GBM negative ANCA associated vasculitis (AAV) patients, and 21 DPPs were enrolled in this study. Among the 21 DPPs aged 68.0 years (59.5, 74.0), there were 11 males and 10 females. The median serum creatinine at diagnosis was 629.0 (343.85, 788.75) μmol/L, and the median eGFR (CKD-EPI) was 7.58 (4.74, 13.77) mL/min. Fifteen cases (71.4 %) underwent initial RRT. After a follow-up of 40.0 (11.0, 73.0) months, 13 out of 21 DPPs (61.9 %) received maintenance RRT, while 49 out of 69 (71.0 %) ANCA negative anti-GBM-GN patients and 124 out of 296 (41.9 %) anti-GBM negative AAV patients received maintenance RRT (P < 0.001). Kaplan-Meier survival analysis showed that DPPs and ANCA negative anti-GBM-GN patients were more likely to progress to ESRD than anti-GBM negative AAV patients (P = 0.001). Among the 21 patients with DPPs, renal survival was significantly better in patients with better initial renal function, including those who did not receive initial RRT (P = 0.003), with lower serum creatinine levels (Cr < 629.0 μmol/L, P = 0.004) and higher eGFR levels (eGFR ≥ 7.60 ml/min, P = 0.005) than those with poor initial renal function. At the end of follow-up, 14 out of 21 DPPs (66.7 %) survived. Survival analysis showed no significant difference among patients in DPPs group, ANCA negative anti-GBM-GN group, and anti-GBM negative AAV group. DPPs and ANCA negative anti-GBM-GN patients were more likely to progress to ESRD than anti-GBM negative AAV patients. In DPPs, the poor renal function at diagnosis might be a risk factor associated with poor renal survival. [ABSTRACT FROM AUTHOR]

Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is relatively rare in children. This article aimed to analyze clinical and renal histology findings and different responses to induction treatment associated with the long-term renal outcomes in children with AAV in a single center. Methods: All pediatric patients with AAV admitted to Tongji Hospital from January 2002 to January 2021 were included in the study. The demographic, clinical, pathological, laboratory, and treatment data and outcomes were collected and analyzed to identify predictors associated with response to induction treatment and progression to end-stage renal disease (ESRD). Results: In total, 48 children with AAV were included in this cohort; 81.25% of them were women, and 91.7% were microscopic polyangiitis (MPA). Kidney involvement was found in 45 patients (93.75%). The most common histopathological subtype was crescentic form in this cohort according to Berden's classification. In total, 34 patients (70.8%) showed eGFR <60 ml/min/1.73 m2 at the time of diagnosis. Complete and partial remission was achieved in 8 patients (16.7%) and 19 patients (39.6%), respectively, following 6-month induction treatment. Half of the patients eventually progressed to ESRD at a mean time of (13.04 ± 15.83) months after diagnosis. The independent predictors of nonremission following induction treatment and progression to ESRD were baseline eGFR <60 ml/min/1.73 m2 and hypertension at diagnosis. Renal survival significantly decreased over time in patients with renal sclerotic subtypes or those with nonremission following induction treatment by Kaplan–Meier curve estimation. Conclusions: Our study demonstrates that women, MPA, and crescentic subtypes are predominant in pediatric AAV in China. Initial renal failure (eGFR <60 ml/min/1.73 m2), hypertension, sclerotic pathological subtype, and nonremission following induction treatment are predictive of long-term renal outcomes. [ABSTRACT FROM AUTHOR]

Objective: Advanced age, need for dialysis and high creatinine values at the time of admission, anti-glomerular basement membrane antibody positivity, pulmonary hemorrhage, and fibrous crescents on pathology are known poor prognostic factors in rapidly progressive glomerulonephritis. Our study aimed to determine the clinical, laboratory, and pathological factors affecting the kidney prognosis in the progression to end-stage kidney failure disease in our rapidly progressive glomerulonephritis patients. Methods: This study was made with 52 patients diagnosed with type 1 and type 3 rapidly progressive glomerulonephritis. The study continued with 37 eligible patients. Results: The patients’ mean age was 52.3 ± 11.06 and 22 (59.4%) were male. In 6 patients (16.2%), anti-glomerular basement membrane positivity, in 11 patients (29.7%), cytoplasmic anti-neutrophilic cytoplasmic antibody positivity, and in 20 patients (54.1%), perinuclear anti-neutrophilic cytoplasmic antibody positivity was detected. Parathyroid hormone value was higher in the perinuclear anti-neutrophilic cytoplasmic antibody-positive group at the time of biopsy (P = .005). Serum sodium levels were lower at the biopsy time in the patient group with a crescent rate above 65% (P = .014). Patients with end-stage kidney failure disease were younger, and their serum sodium levels expressively were lower at the biopsy time (P = .006, P = .02 respectively). When the risk factors affecting end-stage kidney failure disease were examined in multivariate regression analysis, it was observed that while sodium and parathyroid hormone values were not risk factors at the time of biopsy, age (P = .048) and time until diagnosis were risk factors (P = .048). Conclusion: In our study, the risk factors affecting end-stage kidney failure disease progression in rapidly progressive glomerulonephritis patients were age and the time until diagnosis. Kidney biopsy is essential for the kidney prognosis for rapid diagnosis, especially in young patients with suspected rapidly progressive glomerulonephritis. [ABSTRACT FROM AUTHOR]

IntroductionAntineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is relatively rare in children. This article aimed to analyze clinical and renal histology findings and different responses to induction treatment associated with the long-term renal outcomes in children with AAV in a single center.MethodsAll pediatric patients with AAV admitted to Tongji Hospital from January 2002 to January 2021 were included in the study. The demographic, clinical, pathological, laboratory, and treatment data and outcomes were collected and analyzed to identify predictors associated with response to induction treatment and progression to end-stage renal disease (ESRD).ResultsIn total, 48 children with AAV were included in this cohort; 81.25% of them were women, and 91.7% were microscopic polyangiitis (MPA). Kidney involvement was found in 45 patients (93.75%). The most common histopathological subtype was crescentic form in this cohort according to Berden’s classification. In total, 34 patients (70.8%) showed eGFR

Aim: This study aimed to investigate the clinicopathological features and prognosis of immunoglobulin A nephropathy (IgAN) with arterial-arteriolar sclerosis (AS).Methods: Patients with biopsy-proven IgAN from the West China Hospital of Sichuan University were retrospectively enrolled. Clinicopathological features were collected. Patients were categorized based on the presence and the severity of the AS. All the patients were regularly followed-up until a composite end point. The correlation between AS and prognosis of IgAN was assessed.Results: A total of 1,424 patients were recruited and followed for 60.0 ± 28.7 months. Patients with AS tended to have older age, higher blood pressure, heavier proteinuria, higher serum creatinine, uric acid, and total triglyceride (TG). Meanwhile, they were more likely to have a lower estimated glomerular filtration rate (eGFR), hemoglobin, and albumin. At the end of follow-up, 126 patients in the AS group and 47 patients in the non-AS group had reached the composite end point (p < 0.001). AS was associated with the renal outcome (log-rank p < 0.001) and was an independent risk factor for the progression of IgAN (p = 0.049). The severity of AS was associated with renal outcomes (log-rank p < 0.001) and there was a trend that it might serve as an independent risk marker for progression of IgAN. In the subgroup analysis, patients presenting with AS and lower eGFR, albumin, and hemoglobin or higher proteinuria, uric acid, and TG had a significant trend for a shorter time to reach the end point (log-rank p < 0.001).Conclusion: AS was commonly seen in patients with IgAN and was independently associated with the poor prognosis.

Objectives: This study aims to assess the different predictors of renal and patient prognosis in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients with and without renal involvement. Patients and methods: A total of 79 patients (51 males, 28 females; mean age: 57.3±14.2 years; range, 18 to 71 years) with AAV between January 2006 and November 2019 were retrospectively analyzed. Demographic and laboratory data including the complement 3 (C3) serum levels and renal biopsy findings were extracted from the electronic and printed medical records of the hospital registry. Survival, renal survival, remission, and relapse outcomes were analyzed. Results: A total of 35% of the patients with renal involvement progressed to end-stage renal disease (ESRD). The dialysis requirement at the time of admission (hazard ratio [HR]: 21.95 [2.93-164.22]; p=0.003), estimated glomerular filtration rate (eGFR) (HR: 0.97 [0.94-0.99]; p=0.024) and Five-Factor Score (FFS) ≥2 at the time of diagnosis (HR: 3.59 [1.08-11.94]; p=0.037) were the predictors of ESRD. The five-year patient survival rate was 87.1%. The only predictor of mortality was age (HR: 1.07 [1.01-1.14]; p=0.024). The patients with hypocomplementemia (22%) had a lower remission rate (p=0.049), FFS ≥2 at the time of diagnosis (p=0.026), and higher levels of hematuria (p=0.004) and proteinuria (p=0.037). The FFS ≥2 at the time of diagnosis was an independent predictor of relapse (HR: 8.9 [1.02-77.36]; p=0.047). Conclusion: Our study suggests that the baseline renal function and FFS ≥2 at the time of diagnosis are the major prognostic factors for progression to ESRD in AAV patients. In addition, AAV patients with hypocomplementemia may have a lower remission rate. [ABSTRACT FROM AUTHOR]

Abstract Background Incidence of renal dysfunction and risks of progression to end-stage renal disease (ESRD) were reported higher in upper urinary tract urothelial carcinoma (UTUC) than in renal cell carcinoma (RCC) patients after unilateral nephrectomy. Methods Totally 193 renal cancer patients, including 132 UTUC and 61 RCC, were studied to clarify whether the pathological changes of the kidney remnant removed from nephrectomy and the clinical factors might predict the risk of ESRD. Renal tubulointerstitial (TI) score and global glomerulosclerosis (GGS) rate were examined by one pathologist and two nephrologists independently under same histopathological criteria. Results The glomerular filtration rates at the time of surgery were lower in UTUC than RCC groups (p

Background: This investigation was managed to explore whether miR-193a in combination with two podocytes, namely, Wilms tumor type 1 (WT1) and podocalyxin (PODXL), were feasible in estimating onset and prognosis of idiopathic membranous nephropathy (IMN). Methods: We recruited a total of 189 healthy controls and 364 IMN patients, whose urine samples were prepared to measure the expression of miR-193a and PODXL. Meanwhile, renal tissues collected from above-mentioned IMN patients (n = 364) and renal cell carcinoma patients (n = 189) were arranged to determine the expression of WT1. Ultimately, receiver operating characteristic curves were constructed to appraise the performance of miR-193a, WT1, and PODXL in predicting renal survival of IMN patients. Results: The IMN patients were measured with up-regulated miR-193a expression and down-regulated WT1/PODXL expression, when compared with healthy controls (p 3.79 g/24 h)/serum creatinine (>174.63 μmol/L), and declined GFR (≤68.13 mL/min/1.73 m2) were implicated as prominent biomarkers for the poor renal survival of IMN patients (all p

Background: Whether cigarette smoking is associated with the progression of immunoglobulin A nephropathy (IgAN) remains uncertain; therefore, we aimed to evaluate the effect of cigarette smoking on the prognosis of IgAN.Methods: We divided 1239 IgAN patients from West China Hospital of Sichuan University who met the inclusion criteria into smoker (current or former) and non-smoker groups. The endpoint was end-stage renal disease (ESRD: eGFR < 15 mL/min/1.73 m2 or undergoing renal replacement treatment) and/or eGFR decreased by > 50%. Kaplan-Meier, correlation, logistic regression and Cox proportional hazards analyses were performed. The association between cigarette smoking and IgAN was further verified by propensity-score-matched cohort analysis.Results: During the mean follow-up period of 61 months, 19% (40/209) of the smoker group and 11% (110/1030) of the non-smoker group reached the study endpoint (p < 0.001). Multivariate Cox regression analysis revealed that cigarette smoking (hazard ratio (HR) = 1.58; p = 0.043) was an independent risk factor predicting poor renal progression in IgAN, and that IgAN patients with chronic kidney disease (CKD) stage 3-4 were more susceptible to cigarette smoking (p < 0.001). After propensity score matching (PSM), a significant correlation between cigarette smoking and renal outcomes in IgAN patients was seen. Furthermore, Spearman's correlation test revealed that smoking dose was negatively correlated with eGFR (r = 0.141; p < 0.001) and positively related with proteinuria (r = 0.096; p = 0.001).Conclusions: Cigarette smoking is an independent risk factor for IgAN progression, especially for advanced patients. [ABSTRACT FROM AUTHOR]

ObjectiveTo determine the prognostic values of histopathologic classification of myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis and other clinical and laboratory features at the time of presentation on renal and patient survival associated with myeloperoxidase-ANCA-associated glomerulonephritis (MPO-ANCA-GN).MethodsA total of 112 patients diagnosed with MPO-ANCA-GN from October 2005 to December 2018 were enrolled. The baseline clinical characteristics, renal histopathological data, and risk factors predictive of renal and patient survival were retrospectively analyzed.ResultsAll 112 patients underwent renal biopsy. Disease in 32 patients was classified as focal, 26 as mixed, 29 as crescentic, and 25 as sclerotic. Over a median follow-up period of 41.5 months, there were 44 patients dialysis-dependent. The renal survival rate was significantly higher in the focal group than the other groups (p < 0.001) and significantly lower in the sclerotic group (p < 0.05). In addition, disease histopathologically classified as sclerotic (p = 0.044), high serum creatinine level (≥320 μmol/L, p < 0.001), low albumin (

Purpose: Platelet-to-lymphocyte ratio (PLR) was established showing the poor prognosis in several diseases, such as malignancies and cardiovascular diseases. But limited study has been conducted about the prognostic value of PLR on the long-term renal survival of patients with Immunoglobulin A nephropathy (IgAN). Methods: We performed an observational cohort study enrolling patients with biopsy-proven IgAN recorded from November 2011 to March 2016. The definition of composite endpoint was eGFR decrease by 50%, eGFR < 15 mL/min/1.73 m2, initiation of dialysis, or renal transplantation. Patients were categorized by the magnitude of PLR tertiles into three groups. The Kaplan–Meier curves and multivariate Cox models were performed to determine the association of PLR with the renal survival of IgAN patients. Results: 330 patients with a median age of 34.0 years were followed for a median of 47.4 months, and 27 patients (8.2%) had reached the composite endpoints. There were no differences among the three groups (PLR < 106, 106 ≤ PLR ≤ 137, and PLR > 137) in demographic characteristics, mean arterial pressure (MAP), proteinuria, and estimated glomerular filtration rate (eGFR) at baseline. The Kaplan–Meier curves showed that the PLR > 137 group was significantly more likely to poor renal outcomes than the other two groups. Using univariate and multivariate cox regression analyses, we found that PLR > 137 was an independent prognostic factor for poor renal survival in patients with IgAN. Subgroup analysis revealed that the PLR remained the prognostic value for female patients or patients with eGFR less than 60 mL/min/1.73 m2. Conclusions: Our results underscored that baseline PLR was an independent prognostic factor for poor renal survival in patients with IgAN, especially for female patients or those patients with baseline eGFR less than 60 mL/min/1.73 m2. [ABSTRACT FROM AUTHOR]

Objective: To determine the prognostic values of histopathologic classification of myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis and other clinical and laboratory features at the time of presentation on renal and patient survival associated with myeloperoxidase-ANCA-associated glomerulonephritis (MPO-ANCA-GN). Methods: A total of 112 patients diagnosed with MPO-ANCA-GN from October 2005 to December 2018 were enrolled. The baseline clinical characteristics, renal histopathological data, and risk factors predictive of renal and patient survival were retrospectively analyzed. Results: All 112 patients underwent renal biopsy. Disease in 32 patients was classified as focal, 26 as mixed, 29 as crescentic, and 25 as sclerotic. Over a median follow-up period of 41.5 months, there were 44 patients dialysis-dependent. The renal survival rate was significantly higher in the focal group than the other groups (p < 0.001) and significantly lower in the sclerotic group (p < 0.05). In addition, disease histopathologically classified as sclerotic (p = 0.044), high serum creatinine level (≥320 μmol/L, p < 0.001), low albumin (<30 g/L, p = 0.024) and hemoglobin level (<90 g/L, p = 0.044) were associated with a greater risk of ESRD. After follow-up, 70 (62.5%) of 112 patients survived. Old age (≥60 years, p = 0.018) and low serum albumin (<30 g/L, p = 0.006) was significant risk factor for patient survival. Conclusion: Among patients with MPO-ANCA-GN, those with poor renal function, disease histopathologically classified as sclerotic, and lower albumin and hemoglobin levels were risk factors for ESRD, while older age and low serum albumin level were associated with a greater risk for all-cause mortality. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study was to explore the effect of sex on the clinicopathological features and long-term outcomes of IgAN patients. Methods: A total of 1096 adult IgAN patients were divided into male and female groups. Clinicopathological features and risk factors of IgAN patients of different genders were contrasted. The primary endpoint was the combined endpoint of a 50% reduction in estimated glomerular filtration rate (eGFR) and/or end stage renal disease (ESRD: eGFR < 15 mL/min/1.73 m2 or dialysis). The effect of gender on prognosis of IgAN was assessed using Kaplan–Meier and Cox proportional hazards models. Results: In total, 475 male patients and 621 female patients were included in this study. At baseline, male patients had higher values for blood pressure, serum creatinine, urine protein and serum uric acid, as well as lower levels of eGFR. Further analysis indicated that tubular atrophy/interstitial fibrosis (T) lesions and vascular lesions were more frequent in male patients. During the follow-up period of 40.9 ± 24.2 months, kidney survival rates of male IgAN patients were remarkably lower than those of female patients. Using multivariate Cox regression analysis, male gender was identified as an independent risk factor for poor outcomes (β = 0.384, Wald = 4.290, Exp (β) = 1.47, p = 0.038), including hypertension, low eGFR, IgM deposition, arteriosclerosis lesions and T1–T2 lesions. However, male and female patients were characterized by different risk factors. Conclusion: Male patients presented with more severe clinical and pathological changes than female patients. Renal survival rates of male patients were remarkably lower than those of female patients, and male gender was identified as an independent risk factor for poor outcomes. [ABSTRACT FROM AUTHOR]

Background: The efficacy and safety of corticosteroids and immunosuppressive therapy remain controversial for the treatment of immunoglobulin A nephropathy (IgAN). This study aimed to evaluate the effects of corticosteroid and immunosuppressant therapy in Chinese patients with early-stage IgAN whose estimated glomerular filtration rate (eGFR) was ≥45 ml/min/1.73 m2 and proteinuria was ≥1 g/24 h at biopsy.Methods: Patients with biopsy-proven IgAN were retrospectively enrolled from four study centers between 2007 and 2016. Patients were regularly followed up for at least 1 year or until the study end point. Patients were categorized into three treatment groups: supportive care (SC), steroids alone (CS), and steroids plus immunosuppressants (IT). The observed responses to therapy included complete remission (CR), partial remission (PR), no response (NR), and end-stage renal disease (ESRD). The primary end point of the current study was defined as a 50% decline in eGFR and/or ESRD.Results: A total of 715 patients (male 47% and female 53%) were recruited and followed up for 44.69 ± 24.13 months. The observed CR rate was 81.8% with corticosteroids alone (CS), 62.7% with corticosteroids + immunosuppresants (IT), and 37% with supportive care alone (SC). Renal outcomes were remarkably better in the CS group compared with the SC and IT groups (the percentage of patients reaching the end point in each group was 4.6 vs. 14.4 vs. 11.5%, respectively; p = 0.001). Moreover, 36 and 80-month renal survival were significantly better for the CS group (98.3 and 86.4%) than for the IT (94.2 and 82.4%) and SC (94.0 and 51.6%) groups. Early CKD stage also presented with better kidney survival (p < 0.001). Renal survival of CKD stage 1 patients was relatively good regardless of the specific treatment regimen. CS and IT treatment significantly improved renal survival for CKD stage 2 patients when compared with the SC group (p < 0.001 and 0.007, respectively). However, renal survival of CKD stage 3a patients was not impacted by any of the three treatment regimens. Subgroup analysis also showed that renal survival of patients with proteinuria >3.5 g, M1, E0, S1, T0, and C0 was significantly better in the CS group than in the SC and IT groups. A multivariate model showed that hypertension, serum creatinine, E1 lesion, and T1/T2 lesion remained independent predictors of poor renal survival.Conclusions: Immunosuppressive therapy does not have further benefit beyond that provided by steroids. Corticosteroids plus optimal supportive care may further be beneficial in treating early-stage IgAN patients in that it could significantly improve the short-term renal outcome.

Background/Aims: We aimed to explore the associations between clinical parameters and long-term allograft outcomes in transplant glomerulopathy (TG) in a large retrospective cohort with long follow-up. Methods: Clinical and laboratory data at biopsy from 180 cases of TG with an estimated glomerular filtration rate (eGFR)> 15ml/min/1.73m2 from January 2004 to December 2016 at our center were retrospectively analyzed. The main outcome of this study was initiation of replacement therapy or an eGFR declined to < 15 ml/min/1.73m2. Results: During a median follow-up of 5 years (interquartile range 2.6-8.2 years), 117 cases (65.0%) achieved the combined event. Kaplan-Meier method yielded the 1-year and 5-year cumulative renal allograft survival rates after a histopathologic diagnosis of TG were 84% (95% confidence interval [CI] 81-87%) and 33% (95% CI 27–39%) respectively. In univariate analysis, allograft outcome differed significantly by eGFR, proteinuria, blood hemoglobin level, urinary retinol-binding protein (urRBP) and urinary N-acetyl-β-D-glucosaminidase (urNAG) level at the time of biopsy. Multivariate Cox analysis revealed that a higher level of eGFR was the most powerful predictor of allograft survival. Compared with those with eGFR≥60, the hazard ratio (HR) increased from 4.50 (95% CI: 1.03-19.71, p=0.0462) for patients with eGFR between 30 and 59 ml/min/1.73m2 to 9.14 (95% CI 1.97-42.45, P=0.0047) when eGFR decreased to 15 to 29 ml/min/1.73m2. Additionally, proteinuria and higher urRBP values (≥2.85mg/dl) were found to confer much worse survival rates for TG patients in multivariate Cox analysis. Male sex (HR 0.48, P=0.02) and HCV infection (HR 1.78, P=0.0499) were also found to be independent risk factors for worse allograft survival. Conclusion: Five clinical features—impaired renal function, higher proteinuria, higher urRBP level, male sex and HCV infection—are independent predictors of an unfavorable renal allograft outcome. urRBP is a simple and useful parameter that can add invaluable information for the clinical follow-up of patients with TG.

Background/Aims: Treatment of advanced Immunoglobulin A nephropathy (IgAN) patients with estimated glomerular filtration rate (eGFR) below 45 mL/min per 1.73 m2 remains inconsistent. The aim of this study is to compare the effects of corticosteroid and immunosuppressant therapies among these patients. Methods: A total of 201 adult patients with advanced IgAN (eGFR < 45 mL/min/1.73 m2 and proteinuria > 1 g/24h at biopsy) grouped into supportive care (SC), steroids alone (CS), and steroids plus immunosuppressant (IT) groups, were investigated between 30th December 2002 and 30th June 2016. The primary endpoint was the combined endpoint of a 50% decline in eGFR and/or end stage renal disease (ESRD: eGFR < 15 mL/min/1.73 m2 or maintenance renal replacement treatment). Responses to therapy included complete remission (CR: urinary protein excretion < 0.5 g/24h, with eGFR decrease less than 10% baseline), partial remission (PR: proteinuria decrease by > 50% baseline, with eGFR decrease less than 10% baseline), no response (NR: proteinuria decrease < 50% baseline, or eGFR decrease > 10% baseline) and ESRD. Kaplan–Meier and Cox proportional hazards analyses were performed. Results: During the follow-up period (37.2 ± 22.7 months), 6.8% patients in SC group, 25.0% in CS group, and 38.0% in IT group achieved CR or PR, while 78.4%, 62.5% and 49.3% patients in these 3 groups reached primary endpoint respectively (p < 0.001). Three-year renal survival rates in SC and combined immunosuppressive treatment groups (CS and IT groups) were 33.8% vs 51.2% (p = 0.02), and 5-year renal survival rates were 12.2% vs 21.3% (p = 0.1) respectively. Multivariate Cox regression analysis showed that hypertension (HR = 2.44, 95% CI 1.51–3.95; p < 0.001), Scr (HR = 1.01, 95% CI 1.00-1.01; p < 0.001), T1-T2 lesion (HR = 1.99, 95% CI 1.35–2.93; p = 0.001) were independent indicators of poor renal outcome. Conclusion: Immunosuppressive treatment (CS and IT therapy) may improve short-term renal outcome compared with supportive treatment in advanced IgAN patients.

Chronic kidney damage is routinely assessed semiquantitatively by scoring the amount of fibrosis and tubular atrophy in a renal biopsy sample. Although image digitization and morphometric techniques can better quantify the extent of histologic damage, we need more widely applicable ways to stratify kidney disease severity. Methods: We leveraged a deep learning architecture to better associate patient-specific histologic images with clinical phenotypes (training classes) including chronic kidney disease (CKD) stage, serum creatinine, and nephrotic-range proteinuria at the time of biopsy, and 1-, 3-, and 5-year renal survival. Trichrome-stained images processed from renal biopsy samples were collected on 171 patients treated at the Boston Medical Center from 2009 to 2012. Six convolutional neural network (CNN) models were trained using these images as inputs and the training classes as outputs, respectively. For comparison, we also trained separate classifiers using the pathologist-estimated fibrosis score (PEFS) as input and the training classes as outputs, respectively. Results: CNN models outperformed PEFS across the classification tasks. Specifically, the CNN model predicted the CKD stage more accurately than the PEFS model (κ = 0.519 vs. 0.051). For creatinine models, the area under curve (AUC) was 0.912 (CNN) versus 0.840 (PEFS). For proteinuria models, AUC was 0.867 (CNN) versus 0.702 (PEFS). AUC values for the CNN models for 1-, 3-, and 5-year renal survival were 0.878, 0.875, and 0.904, respectively, whereas the AUC values for PEFS model were 0.811, 0.800, and 0.786, respectively. Conclusion: The study demonstrates a proof of principle that deep learning can be applied to routine renal biopsy images.

Abstract Background The prognostic effect of gender on immunoglobulin A nephropathy (IgAN) is not clear. We explored gender-related differences in clinicopathological features and renal outcomes in IgAN. Methods This was a single-centre retrospective study. Patients were divided into two groups according to gender. The clinicopathological features at biopsy and renal outcomes during the follow-up were collected and analysed. Renal outcomes were defined as the doubling of baseline serum creatinine and end-stage renal disease (ESRD) (estimated glomerular filtration rate

Introduction. Serum immunoglobulin A (IgA)/C3 ratio has been reported as a predictor of histological lesions and prognosis in asian patients with IgA nephropathy (IgAN). Since its validity in other populations is unclear, we aimed to evaluate the relationship between IgA/C3 ratio and renal outcome in Caucasian European patients with biopsy-proven IgAN. Methods. We conducted a retrospective, observational study on 95 patients with primary IgAN patients diagnosed between 2010 to 2017 (70% male, age 41 (34 to 49) years, eGFR 39.4 (25.2 to 56.5) mL/min, proteinuria 1.7 (0.8 to 3.0) g/g). The primary study composite end-point was doubling of serum creatinine, ESRD (dialysis or renal transplant) or death, whichever came first. Results. Median follow-up was 30 (95% CI: 27.5 to 32.4) months; 11% developed ESRD, 10% experienced serum creatinine doubling, and 1% died. The endpoint was reached by 21% of the patients. They had lower eGFR, higher proteinuria and hematuria, and lower serum albumin. The distribution in Oxford classes was alike. The AUROC for IgA/C3 ratio was 0.60 (95% CI: 0.45 to 0.74) and generated an optimal cut-off of 2.91 (sensitivity 68%, specificity 55%). The mean event-free survival of the whole cohort was 5.2 (95% CI: 4.7 to 5.8) years. Patients with IgA/C3 ratio < 2.9 had a tendency to better renal survival (P > .05). In Cox proportional hazard ratio model, the independent predictors of a poorer eventfree survival were higher serum creatinine, higher proteinuria and increased IgA/C3 ratio, while renin angiotensin system inhibitors predicted better outcome. Conclusion. Our study reports evidence that supports IgA/C3 ratio as a reasonable predictor of IgAN prognosis in European patients. [ABSTRACT FROM AUTHOR]

Introduction: Lupus nephritis (LN) has a considerable impact on the morbidity and mortality of systemic lupus erythematosus (SLE) patients. Long-term comparative outcome data from the Indian subcontinent on treatment regimens with cyclophosphamide (CYP) and mycophenolate mofetil (MMF) are sparse. We assessed renal and patient survival for these patients in terms of the types of induction – CYP or MMF – and the two maintenance therapies – MMF or azathioprine (AZA). Methods: We retrospectively analysed outcomes of 100 LN patients, 67 treated with CYP (26 class III, 25 class IV, 6 class III + V and 10 class IV + V; 40 Euro lupus regimen and 27 National Institutes of Health regimen) and 33 treated with a MMF-based regimen with steroids between July 2008 and June 2018. Data regarding demographic, clinical and histopathological features and the treatment given to all patients were extracted. Outcomes between the two regimens CYP and MMF were compared in terms of remission, dialysis and patient survival. Results: The clinical characteristics were similar in both groups, except that the activity index was higher in CYP patients (6.13 ± 4.48 vs. 4.61 ± 2.80). However, the chronicity index was similar. The overall remission rate was 70% at the end of induction. The rates of complete remission, partial remission and non-responders in the CYP group were 46.2%, 23.9% and 29.9%, respectively. However, in the MMF group, the corresponding rates were 57.6%, 12.1% and 30.3%, respectively. The 1-, 2-, 3-, 4-, 5- and 10-year patient survival rates in the CYP group were 89.5%, 86.2%, 86.2%, 83.8%, 83.8% and 83.8%, respectively. In the MMF induction group, the corresponding rates were 93.9%, 93.9%, 89%, 89%, 89% and 89%, respectively. At the end of the study, rates of end-stage renal disease in the MMF group and CYP group were 7.5% and 12.1%, respectively. The death-censored and non-censored renal survival rates were also similar in the long term. With regard to maintenance therapy, 3/56 (5.3%) in the MMF group and 7/34 (20.5%) in the AZA group experienced doubling of serum creatinine (p = 0.03). Conclusions: Long-term outcomes in terms of patient and renal survival of LN patients treated with CYP and MMF induction are similar. Doubling of serum creatinine occurred more with AZA-based maintenance therapy than with MMF-based maintenance therapy. Most deaths occurred during induction, and sepsis was the most common cause of death. [ABSTRACT FROM AUTHOR]