The Primary Glomerular Diseases

2017 ◽  
Author(s):  
Richard J. Glassock ◽  
Sanjeev Sethi ◽  
Fernando C. Fervenza
Keyword(s):  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Minimal Change Disease ◽  
Crescentic Glomerulonephritis ◽  
Minimal Change ◽  
C3 Glomerulonephritis ◽  
Focal And Segmental Glomerulosclerosis ◽  
Glomerular Diseases ◽  
Dense Deposit ◽  
Primary Glomerular Diseases

Glomerular disorders in which the manifestations of disease are primarily confined to the kidneys, without multisystem involvement, are not only common but very heterogeneous in terms of pathogenesis and clinical features. Typically, these primary glomerular diseases are characterized according to the findings on renal biopsy, as studied by light, immunofluorescence, and electron microscopy. The principal primary glomerular diseases are minimal change disease, focal and segmental glomerulosclerosis, membranous nephropathy, C3 glomerulonephritis and dense deposit disease, IgA nephropathy, and renal-limited crescentic glomerulonephritis. These clinicopathologic entities are discussed according to epidemiology, clinical features, pathology, pathogenesis (and genetics if appropriate), prognosis, and treatment, emphasizing recent findings.  Key words: C3 glomerulonephritis, dense deposit disease, focal and segmental glomerulosclerosis, glomerulonephritis, IgA nephropathy, membranous nephropathy, minimal change disease, renal biopsy, renal-limited crescentic glomerulonephritis

scholarly journals Establishment of a novel nomogram for the clinically diagnostic prediction of minimal change disease, −a common cause of nephrotic syndrome

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Gaofei Yan ◽  
Guanzhi Liu ◽  
Xuefei Tian ◽  
Lifang Tian ◽  
Hao Wang ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Minimal Change Disease ◽  
Clinical Manifestations ◽  
Minimal Change ◽  
Adult Patients ◽  
Lasso Regression ◽  
Glomerular Diseases ◽  
Laboratory Test Results ◽  
Primary Glomerular Diseases

Abstract Background Minimal change disease (MCD) is one of the major causes of nephrotic syndrome (NS). A confirmed MCD diagnosis mainly depends on renal biopsy at present, which is an invasive procedure with many potential risks. The overall incidence of complications caused by renal biopsy procedures has been reported as approximately 11 and 6.6% outside and within China, respectively. Unfortunately, there is currently no noninvasive procedure or practical classification method for distinguishing MCD from other primary glomerular diseases available. Method A total of 1009 adult patients who underwent renal biopsy between January 2017 and November 2019 were enrolled in this study. Twenty-five parameters extracted from patient demographics, clinical manifestations, and laboratory test results were statistically analysed. LASSO regression analysis was further performed on these parameters. The parameters with the highest area under the curve (AUC) were selected and used to establish a logistic diagnostic prediction model. Results Of the 25 parameters, 14 parameters were significantly different (P < 0.05). MCD patients were mostly younger (36 (22, 55) vs. 41 (28.75, 53)) and male (59% vs. 52%) and had lower levels of diastolic blood pressure (DBP) (79 (71, 85.5) vs. 80 (74, 89)) and IgG (5.42 (3.17, 6.36) vs. 9.38 (6.79, 12.02)) and higher levels of IgM (1.44 (0.96, 1.88) vs. 1.03 (0.71, 1.45)) and IgE (160 (46.7, 982) vs. 47.3 (19, 126)) than those in the non-MCD group. Using the LASSO model, we established a classifier for adults based on four parameters: DBP and the serum levels of IgG, IgM, IgE. We were able to clinically classify adult patients with NS into MCD and non-MCD using this model. The validation accuracy of the logistic regression model was 0.88. A nomogram based on these four classifiers was developed for clinical use that could predict the probability of MCD in adult patients with NS. Conclusions A LASSO model can be used to distinguish MCD from other primary glomerular diseases in adult patients with NS. Combining the model and the nomogram potentially provides a novel and valuable approach for nephrologists to diagnose MCD, avoiding the complications caused by renal biopsy.

scholarly journals Development and Validation of a Discrimination Model Between Primary PLA2R-Negative Membranous Nephropathy and Minimal Change Disease Confirmed by Renal Biopsy

2020 ◽  
Author(s):  
Feng Wu ◽  
Yiding Zhang ◽  
Wen Cui ◽  
Yijun Dong ◽  
Yingyang Geng ◽  
...  
Keyword(s):  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Minimal Change Disease ◽  
Multivariate Logistic Regression Analysis ◽  
Test Group ◽  
Minimal Change ◽  
Clinical Feature ◽  
Specific Marker ◽  
Discrimination Model ◽  
Phospholipase A2 Receptor

Abstract BackgroundMembranous nephropathy (MN) and minimal change disease (MCD) are two common causes leading to nephrotic syndrome (NS). They have similar clinical feature but different treatment strategy and prognosis. M-type phospholipase A2 receptor (PLA2R) is considered as a specific marker of membranous nephropathy. However, its sensitivity is only about 70%. Therefore, there is a lack of effective and noninvasive tools to distinguish PLA2R-negative MN and MCD patients without renal biopsy. We aim to develop a discrimination model using noninvasive parameters for distinguishing the two diseases and support immediate treatment before result of renal biopsy.MethodsA total 949 patients who were pathologically diagnosed as idiopathic MN or primary MCD in the First Affiliated Hospital of Zhengzhou University from January 2017 to August 2019 were enrolled in this study, including 805 idiopathic MN (605 PLAR2-positive MN and 200 PLA2R-negative MN) and 144 primary MCD. Based on the basic information and laboratory examination of 200 PLA2R-negative MN and 144 MCD, we used univariate and multivariate logistic regression analysis to select the relevant variables and develop the discrimination model. ROC curves and calibration curves were used to evaluate the diagnostic ability and calibration ability of the model. The decision curve was used to show the net benefit. We also tested the effectiveness of our model in all 949 patients.ResultsA novel model that included age, albumin, urea, high density lipoprotein, urea and red blood cell count was established for PLA2R-negative MN and MCD. The discrimination model has good differential capability (an AUC of 0.889 in training group and an AUC of 0.920 in test group) and calibration capability. When testing in all 949 patients, our model also showed good discrimination ability for all idiopathic MN and MCD.ConclusionWe constructed a discrimination model with high diagnostic effectiveness for PLA2R-negative MN and MCD. The model could also be used for all idiopathic MN and MCD patients.

scholarly journals Development and validation of a discrimination model between primary PLA2R-negative membranous nephropathy and minimal change disease confirmed by renal biopsy

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Feng Wu ◽  
Yiding Zhang ◽  
Wen Cui ◽  
Yijun Dong ◽  
Yingyang Geng ◽  
...  
Keyword(s):  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Minimal Change Disease ◽  
Treatment Strategies ◽  
Test Group ◽  
Minimal Change ◽  
Specific Marker ◽  
Discrimination Ability ◽  
Discrimination Model ◽  
Phospholipase A2 Receptor

AbstractMembranous nephropathy (MN) and minimal change disease (MCD) are two common causes leading to nephrotic syndrome (NS). They have similar clinical features but different treatment strategies and prognoses. M-type phospholipase A2 receptor (PLA2R) is considered as a specific marker of membranous nephropathy. However, its sensitivity is only about 70%. Therefore, there is a lack of effective and noninvasive tools to distinguish PLA2R-negative MN and MCD patients without renal biopsy. A total 949 patients who were pathologically diagnosed as idiopathic MN or MCD were enrolled in this study, including 805 idiopathic MN and 144 MCD. Based on the basic information and laboratory examination of 200 PLA2R-negative MN and 144 MCD, we used a univariate and multivariate logistic regression to select the relevant variables and develop a discrimination model. A novel model including age, albumin, urea, high density lipoprotein, C3 levels and red blood cell count was established for PLA2R-negative MN and MCD. The discrimination model has great differential capability (with an AUC of 0.904 in training group and an AUC of 0.886 in test group) and calibration capability. When testing in all 949 patients, our model also showed good discrimination ability for all idiopathic MN and MCD.

P0501THE EPIDEMIOLOGICAL FEATURES OF PIRMARY GLOMERULAR DISEASES IN TURKEY: THE MULTICENTER STUDY OF TURKISH SOCIETY OF NEPHROLOGY GLOMERULAR DISEASES (TSN-GOLD) WORKING GROUP

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Aydin Turkmen ◽  
Abdullah Sumnu ◽  
Egemen Cebeci ◽  
Halil Yazici ◽  
Necmi Eren ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Renal Biopsy ◽  
Iga Nephropathy ◽  
Membranous Nephropathy ◽  
Working Group ◽  
Glomerular Diseases ◽  
Nephritic Syndrome ◽  
The Mean ◽  
Primary Glomerular Diseases ◽  
Turkish Society

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

scholarly journals Epidemiological features of primary glomerular disease in Turkey: a multicenter study by the Turkish Society of Nephrology Glomerular Diseases Working Group

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Aydin Turkmen ◽  
Abdullah Sumnu ◽  
Egemen Cebeci ◽  
Halil Yazici ◽  
Necmi Eren ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Renal Biopsy ◽  
Iga Nephropathy ◽  
Membranous Nephropathy ◽  
Working Group ◽  
Glomerular Diseases ◽  
Nephritic Syndrome ◽  
The Mean ◽  
Primary Glomerular Diseases ◽  
Turkish Society

Abstract Background The largest data on the epidemiology of primary glomerular diseases (PGDs) are obtained from the databases of countries or centers. Here, we present the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group. Methods Data of patients who underwent renal biopsy and received the diagnosis of PGD were recorded in the database prepared for the study. A total of 4399 patients from 47 centers were evaluated between May 2009 and May 2019. The data obtained at the time of kidney biopsy were analyzed. After the exclusion of patients without light microscopy and immunofluorescence microscopy findings, a total of 3875 patients were included in the study. Results The mean age was 41.5 ± 14.9 years. 1690 patients were female (43.6%) and 2185 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGD was IgA nephropathy (25.7%) followed by membranous nephropathy (25.6%) and focal segmental glomerulosclerosis (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. The mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. The median proteinuria, serum creatinine, estimated GFR, and mean albumin values were 3300 (IQR: 1467–6307) mg/day, 1.0 (IQR: 0.7–1.6) mg/dL, 82.9 (IQR: 47.0–113.0) mL/min and 3.2 ± 0.9 g/dL, respectively. Conclusions The distribution of PGDs in Turkey has become similar to that in other European countries. IgA nephropathy diagnosed via renal biopsy has become more prevalent compared to membranous nephropathy.

scholarly journals HBV Hepatitis and Related Renal Nephropathies: Pathogenesis and Treatment

2020 ◽  
Vol 4 (2) ◽  
pp. 67-74
Author(s):  
Maurizio Salvadori ◽  
Aris Tsalouchos
Keyword(s):  
Hepatitis B Virus ◽  
Hepatitis B ◽  
Membranous Nephropathy ◽  
Membranoproliferative Glomerulonephritis ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Hbv Infection ◽  
Extrahepatic Manifestations ◽  
Focal And Segmental Glomerulosclerosis ◽  
B Virus

The extrahepatic manifestations of hepatitis B virus (HBV) infection include reactive arthritis, vasculitis (panarteritisnodosa), and primary glomer-ulonephritis (membranous nephropathy, membranoproliferative glomerulonephritis, and, less frequently, IgA nephropathy, focal and segmental glomerulosclerosis, and minimal change disease). No specific histomorphological patterns have been reported in association with HBV infection. The treatment of HBV-related glomerulopathies is essentially antiviral. Peginterferon and nucleos(t)ide drugs are the treatment of choice. Cortico-steroids have been proved to be ineffective (except in panarteritisnodosa), while immunosuppressants can lead to exacerbation of HBV infection.

scholarly journals Synthetic ACTH for Treatment of Glomerular Diseases: A Case Series

2022 ◽  
Vol 9 ◽  
pp. 205435812110669
Author(s):  
Arenn Jauhal ◽  
Bhanu Prasad ◽  
Mathieu Rousseau-Gagnon ◽  
Gabriel Ouellet ◽  
Michelle A Hladunewich
Keyword(s):  
Adverse Events ◽  
Adrenocorticotropic Hormone ◽  
Minimal Change Disease ◽  
Case Series ◽  
Minimal Change ◽  
Lessons Learned ◽  
Focal And Segmental Glomerulosclerosis ◽  
Glomerular Diseases ◽  
Conservative Care ◽  
Segmental Glomerulosclerosis

Rationale: Synthetic adrenocorticotropic hormone (Tetracosactide) has been used in the treatment of refractory glomerular diseases. Literature surrounding the use of this medication is limited to small case series and there is conflicting data on the rate of adverse events associated with this medication. Presenting concerns of the patient: Glomerulonephritis not in remission after at least 6 months of treatment with conservative care. Stable doses of concurrent immunosuppression were permitted. Diagnoses: Membranous nephropathy, IgA nephropathy, minimal change disease, and focal and segmental glomerulosclerosis. Intervention: Intramuscular synthetic adrenocorticotropic hormone (Tetracosactide, Synacthen Depot) with doses of either 1 mg weekly or 1 mg twice weekly. Outcomes: Five of 12 patients had at least a partial remission with Tetracosactide. Median time to response was 6 months for responders. Five of the 12 patients had adverse events documented, 2 of which led to treatment discontinuation. No patients with focal and segmental glomerulosclerosis responded to treatment. Lessons Learned: Higher rate of adverse events than previously reported with synthetic adrenocorticotropic hormone and uncertain treatment efficacy.

scholarly journals Development and Validation of a Between Primary PLA2R-Negative Membranous Nephropathy and Minimal Change Disease Confirmed by Renal Biopsy

2021 ◽  
Author(s):  
Feng Wu ◽  
Yiding Zhang ◽  
Wen Cui ◽  
Yijun Dong ◽  
Yingyang Geng ◽  
...  
Keyword(s):  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Minimal Change Disease ◽  
Test Group ◽  
Minimal Change ◽  
Clinical Feature ◽  
Specific Marker ◽  
Net Benefit ◽  
Discrimination Model ◽  
Phospholipase A2 Receptor

Abstract Background: Membranous nephropathy (MN) and minimal change disease (MCD) are two common causes leading to nephrotic syndrome (NS). They have similar clinical feature but different treatment strategy and prognosis. M-type phospholipase A2 receptor (PLA2R) is considered as a specific marker of membranous nephropathy. However, its sensitivity is only about 70%. Therefore, there is a lack of effective and noninvasive tools to distinguish PLA2R-negative MN and MCD patients without renal biopsy. We aim to develop a discrimination model using noninvasive parameters for distinguishing the two diseases and support immediate treatment before result of renal biopsy.Methods: A total 949 patients who were pathologically diagnosed as idiopathic MN or primary MCD in the First Affiliated Hospital of Zhengzhou University from January 2017 to August 2019 were enrolled in this study, including 805 idiopathic MN (605 PLAR2-positive MN and 200 PLA2R-negative MN) and 144 primary MCD. Based on the basic information and laboratory examination of 200 PLA2R-negative MN and 144 MCD, we used a univariate and multivariate logistic regression to select the relevant variables and develop the discrimination model. ROC curve and calibration curve were used to evaluate the diagnostic ability and calibration ability of the model. The decision curve was used to calculate the net benefit. We also test the effectiveness of our model in all 949 patients.Results: A novel model that included age, albumin, urea, high density lipoprotein, urea and red blood cell count was established for PLA2R-negative MN and MCD. The discrimination model has excellent differential capability (an AUC of 0.889 in training group and an AUC of 0.920 in test group) and calibration capability. When testing in all 949 patients, our model also showed good discrimination ability for all idiopathic MN and MCD.Conclusion: We constructed a discrimination model with high diagnostic effectiveness for PLA2R-negative MN and MCD. The model could also be used for all idiopathic MN and MCD patients.

A case of minimal change disease during pregnancy – Benefits of early diagnosis and use of corticosteroids

2021 ◽  
pp. 1753495X2199021
Author(s):  
Priyanka S Sagar ◽  
Eddy Fischer ◽  
Muralikrishna Gangadharan Komala ◽  
Bhadran Bose
Keyword(s):  
Nephrotic Syndrome ◽  
Early Diagnosis ◽  
Renal Biopsy ◽  
Early Pregnancy ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Risk Of Bleeding ◽  
Increased Risk ◽  
Prompt Diagnosis ◽  
In Pregnancy

Nephrotic syndrome presenting in pregnancy is rare and poses a diagnostic and therapeutic challenge. Timing of renal biopsy is important given the increased risk of bleeding and miscarriage, and the choice of immunosuppression is limited due to the teratogenicity profiles of standard drugs. We report and discuss a case of minimal change disease diagnosed by renal biopsy during early pregnancy and treated with corticosteroids throughout the pregnancy. Prompt diagnosis and treatment of glomerular disease in pregnancy are vital to prevent poor maternal and fetal outcomes.

scholarly journals Clinical manifestations of focal segmental glomerulosclerosis in Japan from the Japan Renal Biopsy Registry: age stratification and comparison with minimal change disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Takaya Ozeki ◽  
Shoichi Maruyama ◽  
Toshiyuki Imasawa ◽  
Takehiko Kawaguchi ◽  
Hiroshi Kitamura ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Clinical Diagnosis ◽  
Clinical Characteristics ◽  
Minimal Change Disease ◽  
Multivariable Analysis ◽  
Laboratory Data ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

AbstractFocal segmental glomerulosclerosis (FSGS) is a serious condition leading to kidney failure. We aimed to investigate the clinical characteristics of FSGS and its differences compared with minimal change disease (MCD) using cross-sectional data from the Japan Renal Biopsy Registry. In Analysis 1, primary FSGS (n = 996) were stratified by age into three groups: pediatric (< 18 years), adult (18–64 years), and elderly (≥ 65 years), and clinical characteristics were compared. Clinical diagnosis of nephrotic syndrome (NS) was given to 73.5% (97/132) of the pediatric, 41.2% (256/622) of the adult, and 65.7% (159/242) of the elderly group. In Analysis 2, primary FSGS (n = 306) and MCD (n = 1303) whose clinical diagnosis was nephrotic syndrome (NS) and laboratory data were consistent with NS, were enrolled. Logistic regression analysis was conducted to elucidate the variables which can distinguish FSGS from MCD. On multivariable analysis, higher systolic blood pressure, higher serum albumin, lower eGFR, and presence of hematuria associated with FSGS. In Japanese nationwide registry, primary FSGS patients aged 18–64 years showed lower rate of NS than those in other ages. Among primary nephrotic cases, FSGS showed distinct clinical features from MCD.

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