Common Type Of Primary Glomerulonephritis Research Articles

Clinical significance of urinary inflammatory biomarkers in patients with IgA nephropathy

BackgroundIgA nephropathy (IgAN) is the most common type of primary glomerulonephritis, although the definitive markers are unknown. We aimed to investigate the clinical significance of urinary cytokines in patients with IgAN.MethodsFrom 2009 to 2018, the patients were divided into three groups: IgAN (n = 191), disease control (n = 53), and normal control (n = 76). We used a multiplex enzyme-linked immunosorbent assay to measure 16 selected urinary inflammatory cytokines, evaluated the correlation between clinical and pathological features following regression analysis on progression.ResultsThe IgAN group exhibited significantly different levels of urinary cytokines compared to the normal control and disease control groups. Urinary levels of B-cell-activating factor, vascular endothelial growth factor receptor-2, monocyte chemoattractant protein-1, C–X–C motif chemokine 10, C–X–C motif ligand 16, epidermal growth factor (EGF), endocan, endostatin, growth/differentiation factor-15 (GDF-15), interleukin-6 (IL-6), mannose-binding lectin, transferrin receptor, and kidney injury molecule-1 were significantly correlated with both the estimated glomerular filtration rate and urine protein–creatinine ratio. In a multivariate Cox regression analysis, urinary EGF (hazard ratio [HR] 0.40, 95% confidence interval [CI] 0.17–0.95, P = 0.04), GDF-15 (HR 2.45, 95% CI 1.01–5.94, P = 0.048), and IL-6 (HR 3.02, 95% CI 1.05–8.64, P = 0.04) were associated with progression in IgAN.ConclusionsUrinary inflammatory biomarkers may serve as alternative predictive biomarkers in patients with IgAN. Further studies are needed to elucidate the physiological mechanisms and confirm the results.

EPIDEMIOLOGY OF BIOPSY CONFIRMED GLOMERULONEPHRITIS IN THE REPUBLIC OF MOLDOVA: PILOT STUDY

Renal biopsy is an important tool for the diagnosis of renal pathologies and for the choice of subsequent treatment tactics. Objectives: To report the epidemiology of glomerulonephritis in the Republic of Moldova, based on histological diagnosis, and set up the premises for the creation of the National Renal Biopsy Registry. Material and methods: The histological results of percutaneous renal ultrasound-guided biopsies, performed from March 30 to February 19, 2023, were evaluated in the Timofei Moșneaga Republican Clinical Hospital, Chisinau. Demographic characteristics, paraclinical parameters (serum creatinine, serum urea, glomerular filtration rate, nictemeral protein), and histological results were analyzed. Results: The outcomes of kidney biopsies performed on fifty-three patients were examined. The prevalence of renal pathologies in young and mature adults was observed, with the average age being 46.2 years. Most of the examined patients were men (71.70%). The main indication for performing renal biopsy was nephrotic syndrome, present in 64.15% of patients. The most common types of primary glomerulonephritis were membranous glomerulonephritis (50% of cases) and membranoproliferative glomerulonephritis (20% of cases). The most frequent types of secondary glomerulonephritis were lupus nephropathy (40%) and renal amyloidosis (30%). Conclusions: This study provides the first image of the current spectrum of glomerular kidney disease in the Republic of Moldova. It also serves as the basis for the development of the National Renal Biopsy Registry, which can serve as a useful resource for health policy development

Identification of IgA autoantibodies targeting mesangial cells redefines the pathogenesis of IgA nephropathy

Immunoglobulin A (IgA) nephropathy (IgAN) is the most common type of primary glomerulonephritis, often progressing to renal failure. IgAN is triggered by IgA deposition in the glomerular mesangium by an undefined mechanism. Here, we show that grouped ddY (gddY) mice, a spontaneous IgAN model, produce serum IgA against mesangial antigens, including βII-spectrin. Most patients with IgAN also have serum anti-βII-spectrin IgA. As in patients with IgAN, IgA+ plasmablasts accumulate in the kidneys of gddY mice. IgA antibodies cloned from the plasmablasts carry substantial V-region mutations and bind to βII-spectrin and the surface of mesangial cells. These IgAs recognize transfected and endogenous βII-spectrin exposed on the surface of embryonic kidney-derived cells. Last, we demonstrate that the cloned IgA can bind selectively to glomerular mesangial regions in situ. The identification of IgA autoantibody and its antigen in IgAN provides key insights into disease onset and redefines IgAN as a tissue-specific autoimmune disease.

Clinical diagnostic performance of common laboratory indicators in primary glomerulonephritis.

Immunoglobulin A nephropathy (IgAN) and membranous nephropathy (MN) are common types of primary glomerulonephritis (PGD). A lack of specific clinical features makes diagnosis difficult. Kidney function indicators have been used for their diagnosis. However, the diagnostic performance of these indicators is undetermined. The purpose of this paper is to evaluate their diagnostic potential. 101 patients with PGD were enrolled, including 50 with MN and 51 with IgAN. The healthy controls included 110 volunteers. The indicators related to kidney function, including TP, ALB, Cre, CysC, eGFR, C1q, Ure, Anti-PLA2R, complement C3, and complement C4 in serum, ACR in urine, and antinuclear antibody profile, IgG staining, IgA staining, IgM staining, C3 staining and C1q staining in tissue samples were evaluated. Statistical differences were found in TP, ALB, Ure, CysC, eGFR, C1q, Anti-PLA2R, complement C3, complement C4 and ACR among the three groups of subjects. ROC analysis showed that Anti-PLA2R and ACR had the highest specificity for identifying IgAN and/or MN from the healthy controls, ACR had the highest sensitivity. The Sp and Se of IgA and IgG in tissue samples for the identification of IgAN and MN were both high. Both IgAN and MN were predicted by anti-PLA2R, especially MN. In tissue samples, MN patients were more likely to be IgG positive and IgAN patients were more likely to be IgA positive. IgAN and MN may be differentiated using serum Anti-PLA2R, tissue IgG, and tissue IgA. Cre is only useful in middle and late stages of GPDs, ACR is an exclusion marker, and CysC and C1q cannot be used to identify MN.

EE646 Systematic Review on Cost and Healthcare Resource Utilization in Immunoglobulin A Nephropathy (IgAN)

Immunoglobulin A nephropathy (IgAN) is the most common type of primary glomerulonephritis. The aim of this systematic review was to assess costs and healthcare resource utilization (HCRU) associated with IgAN.

The association of 5-year therapeutic responsiveness with long-term renal outcome in IgA nephropathy.

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. 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. Among 563 patients from the exploratory cohort, most relapses (13.7%) occurred within 5years after treatment. Using 5-year landmark analysis, among 470 patients, 79 developed ESKD during a median follow-up period of 155months. 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 5years, 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 5years after renal biopsy, which may guide the treatment strategies for patients with IgAN who occasionally relapse after remission.

Urinary Exosomal MicroRNAs as New Noninvasive Biomarkers of IgA Nephropathy.

Immunoglobulin A nephropathy (IgAN) is the most common type of primary glomerulonephritis. It is very important to find new noninvasive biomarkers for the diagnosis and treatment of IgAN. The purpose of this study was to explore the clinical value of urinary exosomal miRNAs in IgAN. In this study, urinary exosomes were isolated from 29 IgAN patients and 29 healthy controls. The miRNA was analyzed by high-throughput sequencing. The expression of hsa-miR-451a and hsa-let-7d-3p was examined by real-time quantitative polymerase chain reaction (RT-qPCR). The diagnostic value of miRNAs was evaluated using receiver operating characteristic (ROC) curves. Here, hsa-miR-451a and hsa-let-7d-3p were upregulated in IgAN patients compared with healthy controls. We evaluated the diagnostic value of hsa-miR-451a and hsa-let-7d-3p using ROC curves; hsa-miR-451a (AUC = 0.805, p = 0.001), hsa-mir-7d-3p (AUC = 0.76, p = 0.0049), and the combination of hsa-miR-451a and hsa-let-7d-3p (AUC = 0.8125, p = 0.0007). Hsa-miR-451a has correlations with Lee's grades (r = 0.511, p = 0.021), and 24-h urinary protein excretion (UPE; r = 0.557, p = 0.011). Hsa-let-7d-3p showed correlations with Lee's grades (r = 0.6, p = 0.005), UPE (r = 0.518, p = 0.019), serum creatinine (r = 0.564, p = 0.01), and estimated glomerular filtration rate (r = -0.532, p = 0.016). According to the Oxford classification, for hsa-miR-451a, S0 had lower levels than S1 (p = 0.016); for hsa-mir-7d-3p, M0 had lower levels than M1 (p = 0.05). These findings suggest that hsa-miR-451a and hsa-let-7d-3p may serve as noninvasive biomarkers for the evaluation of IgAN.

New COL4A5 mutation in IgA nephropathy

PurposeIgA nephropathy (IgAN) is the most common type of primary glomerulonephritis and a leading cause of chronic kidney disease (CKD) and end-stage kidney disease (ESKD). Recently, some case reports have...

Effect of Astragalus membranaceus extract on IgA nephropathy: a case of renal function improvement during one-and-a-half-year follow-up

Immunoglobulin A nephropathy is one of the most common type of primary glomerulonephritis globally. Its pathogenesis remains unclear. It is a slowly progressive disease that may eventually lead to end-stage renal disease. Although the angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers, and immunosuppressive drugs are regarded as the standard treatments, the optimal approach to the treatment of IgA nephropathy is still uncertain. Astragalus membranaceus has been widely used in traditional Chinese medicine for cardiovascular disorder, kidney disease, hepatitis, and some skin diseases. It had been widely used in treating various renal diseases including IgA nephropathy. In this case report, we present a 34-year-old Myanmar female of Karen ethnic with IgA nephropathy. She was treated with Astragalus membranaceus together with losartan and other integrative medicines. Her renal function and proteinuria improved during the one-a-half-year follow-up. This case report highlights the improvement effect of Astragalus membranaceus in integrative way for the treatment of IgA nephropathy.

MON-053 CLINICAL AND PATHOLOGICAL CHARACTERISTICS OF IgA NEPHROPATHY WITH AKD

IgA nephropathy is one of the most common types of primary glomerulonephritis worldwide.It was found that the creatinine of IgA nephropathy could increase in a short time even reaching the diagnostic standard of acute kidney disease（AKD）in clinical work. However, the factors for the occurrence in these patients were unclear. Here,we did a research to explore the clinical features and kidney pathological characteristics of IgA nephropathy patients with AKD and the related factors influencing its recovery. Patients between 2011 and 2016 taken from the First Affiliated Hospital of Zhengzhou University were enrolled in the study.The number of renal glomeruli in all cases was more than or equal to 10.The secondary IgA nephropathy such as systemic lupus erythematosus, purpura nephritis, chronic liver diseases was not included.The 2012 KDIGO guidelines were adopted as the criteria to screen the IgA nephropathy patients with AKD. The baseline data, clinical and renal pathological data were collected.According to the degree of the decline of estimated glomerular filtration rate(eGFR), the IgA nephropathy patients with AKD were divided into three groups with the eGFR decreased by 35-50% in group A, 51-75% in group B, and 76% or more in group C.Then according to the recovery degree of the renal function, the patients were divided into two groups with the recovery group (fully recovery of renal function or partial recovery) and the non-recovery group(no obvious change of renal function or progression of renal function).The clinical features and kidney pathological characteristics of the three groups and the related factors affecting their recoveries were analyzed. A total of 1855 patients were included of which 128 patients occurred to AKD, accounting for 6.9%.There were significant differences in calcium, phosphorus,hemoglobin,albumin,urea nitrogen and urine leukocyte in the three groups (p < 0.05).We followed up the IgA nephropathy patients with AKD for 6 months and found that the creatinine level of 26 patients was not better or even progressed compared with the peak of creatinine during hospitalization, while the creatinine level of 102 patients was lower or completely restored to the baseline.High blood pressure and large amount of proteinuria are the factors that affect the recovery of renal function of AKD.At the same time, we take these patients for a long-term follow-up with the average follow-up time to 3.8 years with the result that 35 patients entered end-stage renal disease(ESRD) or died. The degree of decreased glomerular filtration rate in IgA nephropathy patients with AKD is related to clinical and pathological characteristics and IgA nephropathy patients occurred with AKD are more likely to enter the ESRD.

The Value of Serum IgA in the Diagnosis, Clinical and Pathological Evaluation of Patients with IgA Nephropathy Found During Physical Examination in China.

The goal was to study the role of serum IgA in patients with IgA nephropathy (IgAN) found during physical examination, and to explore its value in diagnosis, assessment of pathological injury, and clinical prediction of IgAN. The study included 457 patients who were hospitalized between January 2010 and June 2018 due to physical abnormalities and diagnosed with kidney disease via renal biopsy. Renal histopathology was quantified according to Katafuchi semi-quantitative standards, while the IgAN patients were also scored according to Lee's grading system. The average age of the 457 patients was 39.62 ± 13.52 years when abnormalities were found during physical examination. IgAN (202 cases, 46.12%) was the most common type of primary glomerulonephritis in the 457 patients. Of the IgAN patients, 75.25% (152 cases) were under 45 years old at the time of abnormal physical examination and IgAN patients were significantly younger than non-IgAN patients. There was a significant difference in the gender ratio between IgAN patients and non-IgAN patients (χ2 = 4.24, p = 0.039). In IgAN patients, the proportion of male patients, serum creatinine (SCr), the glomerular lesion and tubulointerstitial scores, and serum IgA were statistically higher than in non-IgAN patients with other types of primary glomerulonephritis; however, MDRD-GFR was lower. The ROC curve of serum IgA in the diagnosis of IgAN showed the AUC was 0.602. One hundred forty-seven cases (72.77%) were Lee's III - V grade. The proportion of patients who were at Lee's III - V grades in the normal serum IgA group (184 cases) was higher than that of the elevated serum IgA group (18 cases). There were no significant differences in gross hematuria, proteinuria, MDRD-GFR, SCr, and hypertension between the two groups. Serum IgA may be of little value in the diagnosis of patients with IgA nephropathy detected via physical examination. The level of serum IgA may have predictive value in evaluating Lee's pathological damage in IgAN patients.

A case of IgA nephropathy with deep venous thrombosis in the mesentery and lower extremities

IgA nephropathy (Berger disease) is a disorder in which there is prominent IgA deposition in the glomerular mesangial area, and is the most common type of primary glomerulonephritis (1), receiving more and more attention (2-8). With diverse clinical manifestations, it has its own characteristic type of glomerular mesangial lesions (9).

Changes in the diagnosis of glomerular diseases in east China: a 15-year renal biopsy study

Background: There have been some gradual changes in the distribution of renal biopsy pathological diagnoses during recent years. This study aimed to show changes in renal disease prevalence in China by investigating 10 patients diagnosed at our Kidney Disease Centre during the last 15 years.Methods and results: All patients aged 15-year-old or older who underwent renal biopsy at the First Affiliated Hospital, Zhejiang University, from 2001 to 2015 were enrolled. There were 5 common types of primary glomerulonephritis: IgA nephropathy (IgA N), membranous nephropathy (MN), mesangial progressive glomerulonephritis (MsPGN), minimal change disease (MCD), and focal segmental glomerulosclerosis (FSGS), which represented 50%, 16.8%, 15.9%, 8.1% and 2.5% of total cases, respectively. IgA nephropathy was the most common type of primary glomerulonephritis (PGN).Conclusions: Our results mostly showed a new trend that the diagnosis of IgA nephropathy was not increasing and the prevalence of membranous nephropathy had increased, becoming the second most common type of primary glomerulonephritis.Key POINTSDistinguished with other domestic studies, IgA nephropathy did not show a trend of continuous growth although it still had about the half proportion of PGN, whereas membranous nephropathy kept rising and became the second common PGN.Concerning SGN, LN peaked in the younger-age and middle-age groups with a significant female prevalence, DN, BANS and SV had a male predominance peaking in the middle-age and old-age groups.

Recent advances in the physiopathology of IgA nephropathy

Immunoglobulin A nephropathy or Berger's disease is the most common type of primary glomerulonephritis, which is characterized by IgA1-containing immune-deposits in the glomerular mesangium. Microscopic haematuria and proteinuria are the most common presentations. Mesangial cell proliferation with IgA deposition is found on renal biopsy. Mechanims of the disease implicate at least four key molecules have been implicated in immune complex formation: galactose-deficient IgA1, autoantibodies anti-galactose-deficient-IgA1, soluble CD89 (Fc receptor for IgA) and the CD71 mesangial IgA receptor (transferrin receptor). These factors associated with environmental factors (antigens, food and microbiota) are correlated with disease progression and recurrence after transplantation. This review exploits recent data on the role of these molecular players of the disease, which may improve future therapeutic management of immunoglobulin A nephropathy.

The Spectrum of Biopsy-Proven Glomerular Disease in China

Background:Chronic kidney disease has become a leading public health concern in China, as it is associated with increased morbidity, mortality, and costs. However, the overall situation regarding common glomerular diseases in China remains unclear. Hence, the aim of this study was to assess the national profile of the common types of glomerulonephritis in China.Methods:We searched Medline, Embase, Cochrane Library, CNKI, SinoMed, VIP, and Wanfang databases for English and Chinese language articles from inception to September 2017. We also collected potentially relevant studies and reviews using a manual search. The following words in combinations are as keywords: “renal biopsy”, “kidney pathological diagnosis”, and “spectrum of pathological types”.Results:We identified 23 studies involving 176,355 patients from 15 provinces/cities in China. The detection rates of primary glomerulonephritis (PGN) and secondary glomerulonephritis (SGN) were 0.740 and 0.221, respectively. Over the past 30 years, the top five types of PGN were immunoglobulin A nephropathy (IgAN; 24.3%), mesangial proliferative glomerulonephritis (MsPGN; 10.5%), membranous nephropathy (MN; 12.6%), minimal change disease (MCD; 9.8%), and focal segmental glomerulosclerosis (FSGS; 4.6%), and the top four types of SGN were lupus nephritis (LN; 8.6%), Henoch-Schönlein purpura glomerulonephritis (4.1%), hepatitis B virus-associated glomerulonephritis (HBV-GN; 2.6%), and diabetic nephropathy (DN; 1.6%). The proportion of MN, MCD, HBV-GN, and DN tended to increase, while those of IgAN, MsPGN, FSGS, and LN tended to drop.Conclusions:Although the incidence of SGN is increasing gradually, PGN is still the leading form of kidney disease in patients undergoing renal biopsies in China. IgAN and LN are the most common types of PGN and SGN, respectively. Differences between regions are related to various factors such as nationality, environment, and diet. Furthermore, unified standards and norms for evaluating renal biopsies are urgently needed.

Differential expression of urinary exosomal microRNAs in IgA nephropathy

Immunoglobulin A nephropathy (IgAN) is the most common type of primary glomerulonephritis in the world. Reliable biomarkers are required for the non-invasive diagnosis and monitoring of IgAN. This study aims to investigate the difference in urinary exosomal microRNA (miRNA) expression profiles between patients with IgA nephropathy (IgAN) and healthy controls, which may provide clues to identify novel potential non-invasive miRNA biomarkers for renal diseases. Urine samples were collected from eighteen healthy controls and eighteen patients with IgAN. Differential centrifugation was performed to isolate exosomes from urine samples. High-throughput sequencing and real-time quantitative polymerase chain reaction (RT-qPCR) were sequentially used to screen and further validate miRNA expression profiles in urinary exosomes of patients with IgAN in two independent cohorts. Urinary exosomes were successfully isolated to obtain exosomal miRNAs. MiR-215-5p and miR-378i were significantly upregulated in urinary exosomes of patients with IgAN compared with healthy controls (P<.01), while miR-29c and miR-205-5p were significantly downregulated (P<.05). MiR-215-5p, miR-378i, miR-365b-3p and miR-135b-5p were found to have altered expression in patients with IgAN from validation cohorts, which was consistent with the high-throughput sequencing analysis. This study suggests that there is a significant difference in urinary exosomal miRNA profiles between patients with IgAN and healthy controls. These exosomal miRNAs, such as miR-29c, miR-146a and miR-205 may potentially serve as novel non-invasive biomarkers for IgAN.

MiR-100-3p and miR-877-3p regulate overproduction of IL-8 and IL-1β in mesangial cells activated by secretory IgA from IgA nephropathy patients

IgA nephropathy (IgAN) is the most common type of primary glomerulonephritis, characterized by mesangial deposition of pathogenic IgA and the injury to mesangial cells. Our previous studies indicate that secretory IgA (SIgA) plays an important role in the pathogenesis of IgAN, and miR-16 is involved in destructive process in mesangial cells mediated by the SIgA from IgAN patients. Our current study aimed to study the role of miRNAs in the effect of SIgA from IgAN patients on mesangial cells. MicroRNA microarray and cytokines assay were performed to obtain the differential microRNAs expression profile in human renal mesangial cells stimulated by SIgA from IgAN patients (P-SIgA) with the cells treated by SIgA from healthy subjects (N-SgA) as control. The microRNAs with the most significant differences in microarray analysis were validated by quantitative RT-PCR. Among them, miR-100-3p and miR-877-3p were selected to predict target gene related to cytokines detecting in this study. Fifty-six differentially expressed microRNAs were chosen and 17 microRNAs with the most prominent changes were validated. Compared with N-SIgA, P-SIgA increased the production of interleukin (IL)-1β, IL-8, monocyte chemotactic protein-1 and transforming growth factor-β1. In addition, we for the first time demonstrated that over-production of IL-8 induced by the SIgA was regulated by down-expression of miR-100-3p in mesangial cells. Similarly, IL-1β over-production was regulated by down-expression of miR-877-3p. Our findings represent a pathogenic microRNAs expression profiling in human mesangial cells activated by P-SIgA. Furthermore, we provide a new explanation characterizing the molecular mechanism responsible for the regulation of IL-1β and IL-8 production in P-SIgA-triggered mesangial cells.

A clinicopathologic study of glomerular disease: A single-center, five-year retrospective study from Northwest India.

Studies published from centers across India have reported different and contradicting patterns of glomerular disease. In this retrospective study, we report our experience from a Tertiary Care Center in Northwest India. A total of 702 renal biopsies performed between 2008 and 2013 were reviewed of which 80 were excluded from the study because of having insufficient records or if the biopsies were taken from an allograft. The study included 411 males (66.1 %) and 211 females (33.9%) with an age range of 12-70 years (mean 30.34 ± 7.04 years). Majority of the biopsies (93.9%) showed some form of glomerulonephritis (GN), either primary (79.4%) or secondary glomerular disease (SGD) (14.5%). Minimal change disease (MCD) was the most common type of primary GN (26.5% of primary GN), followed by membranous nephropathy (MN; 18.8%) and focal and segmental glomerulosclerosis (FSGS; 13.2%). Lupus nephritis (LN) was the most frequent SGD (52.2% of secondary GN). Amyloidosis was found in 41.1% and diabetic glomerulosclerosis in 4.4%. LN was also the second most common diagnosis in females after MCD, seen in 19.4% of females. MCD followed by membranoproliferative GN and diffuse proliferative GN were the most common entities in individuals <20 years of age. In the 20-39 years age group, MN was the most common pathology seen. MN was again the most common pathology seen in patients aged above 40 years followed by amyloidosis and FSGS. In this study, MCD was the most common primary GN observed overall from this part of India. MN was the most common GN in individuals above 20 years of age presenting with the nephrotic syndrome. The geographical and regional differences in the pattern of GNs point to the necessity of having a central biopsy registry.

Spectrum of clinico-pathological profile and treatment response in children with nephrotic immunoglobulin a nephropathy.

Immunoglobulin A nephropathy (IgAN) is one of the most common types of primary glomerulonephritis in the world. Nephrotic syndrome is an uncommon presentation of IgAN. To evaluate the clinico-pathological features and treatment response of nephrotic IgAN, we prospectively studied 20 nephrotic children with biopsy-proven IgAN at our center from August 2009 to December 2012. The histopathological characterization of IgAN was carried out with the HAAS classification. The demographic profile, clinical presentation, initial laboratory, biopsy findings and treatment response were analyzed. The mean age was 6.7 years. The most common indication of renal biopsy was steroid-dependent nephrotic syndrome associated with microscopic hematuria (65%) and hypertension (25%). The majority of cases were classified as HAAS-III stage. Fifteen cases responded to oral cyclosporine-A, four cases to oral cyclophosphamide and one to mycophenolate mofetil. Complete remission of the nephrotic syndrome was achieved in 90% (18/20) cases within 3 months of initiation of therapy. Two cases that had partial remission were in the HASS-II and III stages. We conclude that the majority of children with nephrotic IgAN responded to oral cyclosporine-A. However, a larger cohort and longer duration follow-up are required to confirm our results.

Capsaicin induces high expression of BAFF and aberrantly glycosylated IgA1 of tonsillar mononuclear cells in IgA nephropathy patients

BackgroundIgA nephropathy (IgAN) is the most common type of primary glomerulonephritis in the world. Hot pepper is the most favorite vegetable for Chinese in Hunan and Sichuan provinces. It can be assumed that capsaicin, the active ingredient of hot pepper, is a possible risk factor in diet in the pathogenesis of IgAN. Methods22 subjects, including 11 IgAN patients and 11 non-IgAN patients were enrolled in this study. Tonsillar mononuclear cells were isolated and cultured for 3days with or without capsaicin. ResultsIn the absence and presence of capsaicin, the BAFF expression and IgA1 secretion were higher in IgAN patients than that in non-IgAN patients, meanwhile, the gene expression of C1GALT1 and Cosmc and IgA1 O-glycosylation level were significantly lower. In IgAN group, coincubated with capsaicin, IgA1 and BAFF secretion and BAFF expression by TMCs were significantly higher than that without capsaicin, furthermore, the level of mRNA encoding C1GALT1 and Cosmc and the level of IgA1 O-glycosylation were evidently lower. ConclusionCapsaicin may induce IgA1 secretion by activating BAFF expression, and bring to aberrantly IgA1 O-glycosylation by suppressing C1GALT1 and Cosmc expression. Therefore, to limit the consumption of hot pepper would be beneficial to patients with IgA nephropathy.