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

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

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

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

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

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

scholarly journals Leflunomide Plus Low-dose Prednisone in Patients with Progressive IgA Nephropathy: A Multicenter, Prospective, Randomized, Open-labelled and Controlled Trial

2020 ◽  
Author(s):  
Zhaohui Ni ◽  
Zhen Zhang ◽  
Zanzhe Yu ◽  
Fuming Lu ◽  
Changlin Mei ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Low Dose ◽  
Controlled Trial ◽  
Efficacy And Safety ◽  
Urine Protein Excretion ◽  
Protein Excretion ◽  
Risk Of Progression ◽  
The Difference ◽  
Dose Prednisone

Abstract Background: This trial was designed to assess the efficacy and safety of Leflunomide (LEF) plus low-dose prednisone for the treatment of progressive IgA nephropathy (IgAN). Methods: We did a prospective, randomized, open-labelled, multicenter, controlled trial, comprised of 3-month run-in, 12-month treatment and 12-month follow-up phases. After 3-month run-in phase, patients with biopsy-confirmed IgAN at a risk of progression were randomly allocated to LEF plus low-dose prednisone (LEF group) or conventionally accepted-dose prednisone (prednisone group). Our primary outcome was 24h urine protein excretion(UPE) and secondary outcomes were serum albumin(sALB), serum creatinine(Scr), and eGFR. Safety was evaluated in all patients who received the trial medications. Results:108 patients (59 in LEF group, 49 in prednisone group) were enrolled and finished their treatment and follow-up periods. The difference in baseline data between the two groups was comparable. Compared with baseline, both groups showed significant decrease in 24h UPE(p<0.01) and increase in sALB (p<0.01), with stable Scr and eGFR throughout the 12-month treatment period. What’s more, these effects sustained through the 12-month follow-up period. However, there was no difference in 24h UPE, sALB, Scr and eGFR between the two groups (P>0.05). At 12 months, difference of overall response rate, relapsing rate and incidence of adverse events between the two groups was not significant. Conclusions: The efficacy and safety of LEF plus low-dose prednisone and conventionally accepted-dose prednisone in treatment of progressive IgAN are comparable. Trial registration: The trial is registered at isrctn.org with the ISRCTN97636235 on July 28, 2006.

scholarly journals RETROSPECTIVE STUDY OF HISTOLOGICAL ANALYSIS AND ITS CORRELATION WITH CLINICAL PRESENTATION AND OUTCOME OF IGA NEPHROPATHY FROM A TERTIARY CENTRE OF GUWAHATI ASSAM.

2019 ◽  
Vol 3 (2) ◽  
Author(s):  
Manjuri Sharma ◽  
Manzoor Ahmad Parry ◽  
Hamad Jeelani ◽  
Pranab Jyoti Mahanta
Keyword(s):  
Retrospective Study ◽  
Iga Nephropathy ◽  
Clinical Presentation ◽  
Interstitial Fibrosis ◽  
Clinical Manifestations ◽  
Tubular Atrophy ◽  
Glomerular Diseases ◽  
Mesangial Hypercellularity ◽  
Endocapillary Hypercellularity

Background: IgA nephropathy (IgAN) is one of the most common glomerular diseases with varied presentations. We aimed to study clinical presentation and outcome of IgAN and correlate with histopathology at the time of presentation. Methods: This is a retrospective study in which we analyzed kidney biopsy data, clinical manifestations and outcome of 137 patients with a diagnosis of primary IgAN from 2012 to 2016. Kidney biopsies were reviewed as per Oxford classification assessing mesangial hypercellularity, endocapillary hypercellularity, segmental sclerosis/adhesion, tubular atrophy/interstitial fibrosis. Correlation analysis was done for biopsy findings and clinical presentation/outcome. P score less than 0.05 was taken as significant. Results: Mean age for presentation was 27.35 years with 83 males and 54 females. Asymptomatic urinary abnormality was the most common clinical presentation (28.5%). Mean serum creatinine was 2.23 ± 2.06mg/dl with mean proteinuria of 1.49 ± 1.43g/day. Mesangial hypercellularity (M) and Endocapillary hypercellularity (E) lesions were significantly associated with proteinuria at the time of biopsy (p=0.02& 0.04 respectively). Segmental glomerulosclerosis (S) and tubular atrophy (T) were significantly associated with eGFR and mean arterial pressure at the time of biopsy. Mean time of follow up was 1.6 years. M1, E0, S1, T0 were the most common lesions. M, S and T lesions in biopsy were significantly associated with decrease in GFR at the end of follow up. Conclusion: In our study, most common presentation of IgAN was AUA with rarity of macroscopic hematuria. M, S and T lesions were associated with decreased GFR on follow up. Key words: IgA Nephropathy, Nephrotic Syndrome, Oxford MEST classification

scholarly journals Long term outcome of immunoglobulin A (IgA) nephropathy: A single center experience

PLoS ONE ◽  
2021 ◽  
Vol 16 (4) ◽  
pp. e0249592
Author(s):  
Rozita Mohd ◽  
Nur Ezzaty Mohammad Kazmin ◽  
Rizna Abdul Cader ◽  
Nordashima Abd Shukor ◽  
Yin Ping Wong ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Primary Outcome ◽  
Interstitial Fibrosis ◽  
Univariate Analysis ◽  
Tubular Atrophy ◽  
Term Survival ◽  
Long Term Outcome ◽  
Time Average

Introduction IgA nephropathy (IgAN) has a heterogeneous presentation and the progression to end stage renal disease (ESRD) is often influenced by demographics, ethnicity, as well as choice of treatment regimen. In this study, we investigated the long term survival of IgAN patients in our center and the factors affecting it. Methods This study included all biopsy-proven IgAN patients with ≥ 1year follow-up. Patients with diabetes mellitus at diagnosis and secondary IgAN were excluded. Medical records were reviewed for demographics, clinical presentation, blood pressure, 24-hour urine protein, serum creatinine, renal biopsy and treatment received. The primary outcome was defined as combined event of 50% estimated glomerular filtration rate (eGFR) reduction or ESRD. Results We included 130 (74 females; 56 males) patients of mean age 38.0 ± 14.0 years and median eGFR of 75.2 (interquartile range (IQR) 49.3–101.4) ml/min/1.73m2. Eighty-four (64.6%) were hypertensive at presentation, 35 (26.9%) had nephrotic syndrome and 57 (43.8%) had nephrotic range proteinuria (NRP). Median follow-up duration was 7.5 (IQR 4.0–13.0) years. It was noted that 18 (13.8%) developed ESRD and 34 (26.2%) reached the primary outcome. Annual eGFR decline was -2.1 (IQR -5.3 to -0.1) ml/min/1.73m2/year, with median survival of 20 years. Survival rates from the combined event (50% decrease in eGFR or ESRD) at 10, 20 and 30 years were 80%, 53% and 25%, while survival from ESRD were 87%, 73% and 65%, respectively. In the univariate analysis, time-average proteinuria (hazard ratio (HR) = 2.41, 95% CI 1.77–3.30), eGFR <45ml/min/1.73m2 at biopsy (HR = 2.35, 95% CI 1.03–5.32), hypertension (HR = 2.81, 95% CI 1.16–6.80), mean arterial pressure (HR = 1.02, 95% CI 1.01–1.04), tubular atrophy/interstitial fibrosis score (HR = 3.77, 95% CI 1.84–7.73), and cellular/fibrocellular crescent score (HR = 2.44, 95% CI 1.19–5.00) were found to be significant. Whereas only time-average proteinuria (TA-proteinuria) remained as a significant predictor in the multivariate analysis (HR = 2.23, 95% CI 1.57–3.16). Conclusion In our cohort, TA-proteinuria was the most important predictor in the progression of IgAN, irrespective of degree of proteinuria at presentation.

Identifying patients with CKD risk at the time of nephrectomy: When to initiate nephrology consult

2021 ◽  
pp. 239936932110319
Author(s):  
Yihe Yang ◽  
Zachary Kozel ◽  
Purva Sharma ◽  
Oksana Yaskiv ◽  
Jose Torres ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multivariate Analysis ◽  
Radical Nephrectomy ◽  
Mixed Model ◽  
Linear Mixed Model ◽  
Interstitial Fibrosis ◽  
Kidney Neoplasm ◽  
Tubular Atrophy ◽  
Independent Risk Factors

Introduction: The prevalence of chronic kidney disease (CKD) is high among kidney neoplasm patients because of the overlapping risk factors. Our purpose is to identify kidney cancer survivors with higher CKD risk. Methods: We studied a retrospective cohort of 361 kidney tumor patients with partial or radical nephrectomy. Linear mixed model was performed. Results: Of patients with follow-up >3 months, 84% were identified retrospectively to fulfill criteria for CKD diagnosis, although CKD was documented in only 15%. Urinalysis was performed in 205 (57%) patients at the time of nephrectomy. Multivariate analysis showed interstitial fibrosis and tubular atrophy (IFTA) >25% ( p = 0.005), severe arteriolar sclerosis ( p = 0.013), female gender ( p = 0.024), older age ( p = 0.012), BMI ⩾ 25 kg/m2 ( p < 0.001), documented CKD ( p < 0.001), baseline eGFR ⩽ 60 ml/min/1.73 m2 ( p < 0.001), and radical nephrectomy ( p < 0.001) were independent risk factors of lower eGFR at baseline and during follow-up. Average eGFR decreased within 3 months post nephrectomy. However, patients with different risk levels showed different eGFR time trend pattern at longer follow-ups. Multivariate analysis of time × risk factor interaction showed BMI, radical nephrectomy and baseline eGFR had time-dependent impact. BMI ⩾ 25 kg/m2 and radical nephrectomy were associated with steeper eGFR decrease slope. In baseline eGFR > 90 ml/min/1.73 m2 group, eGFR rebounded to pre-nephrectomy levels during extended follow-up. In partial nephrectomy patients with baseline eGFR ⩾ 90 ml/min/1.73 m2 ( n = 61), proteinuria ( p < 0.001) and BMI ( p < 0.001) were independent risk factors of decreased eGFR during follow up. Conclusions: As have been suggested by others and confirmed by our study, proteinuria and CKD are greatly under-recognized. Although self-evident as a minimum workup for nephrectomy patients to include SCr, eGFR, urinalysis, and proteinuria, the need for uniform applications of this practice should be reinforced. Non-neoplastic histology evaluation is valuable and should include an estimate of global sclerosis% (GS) and IFTA%. Patients with any proteinuria and/or eGFR ⩽ 60 at the time of nephrectomy or in follow-up with urologists, and/or >25% GS or IFTA, should be referred for early nephrology consultation.

scholarly journals In Patients with Membranous Lupus Nephritis, Exostosin-Positivity and Exostosin-Negativity Represent Two Different Phenotypes

2021 ◽  
pp. ASN.2020081181 ◽  
Author(s):  
Aishwarya Ravindran ◽  
Marta Casal Moura ◽  
Fernando C. Fervenza ◽  
Samih H. Nasr ◽  
Mariam P. Alexander ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Kidney Biopsy ◽  
Interstitial Fibrosis ◽  
Study Cohort ◽  
Lower Serum ◽  
Tubular Atrophy ◽  
Novel Proteins ◽  
Biopsy Specimens ◽  
Membranous Lupus Nephritis

BackgroundIn patients with secondary (autoimmune) membranous nephropathy, two novel proteins, Exostosin 1 and Exostosin 2 (EXT1/EXT2), are potential disease antigens, biomarkers, or both. In this study, we validate the EXT1/EXT2 findings in a large cohort of membranous lupus nephritis.MethodsWe conducted a retrospective cohort study of patients with membranous lupus nephritis, and performed immunohistochemistry studies on the kidney biopsy specimens against EXT1 and EXT2. Clinicopathologic features and outcomes of EXT1/EXT2-positive versus EXT1/EXT2-negative patients were compared.ResultsOur study cohort included 374 biopsy-proven membranous lupus nephritis cases, of which 122 (32.6%) were EXT1/EXT2-positive and 252 (67.4%) were EXT1/EXT2-negative. EXT1/EXT2-positive patients were significantly younger (P=0.01), had significantly lower serum creatinine levels (P=0.02), were significantly more likely to present with proteinuria ≥3.5 g/24 h (P=0.009), and had significantly less chronicity features (glomerulosclerosis, P=0.001 or interstitial fibrosis and tubular atrophy, P<0.001) on kidney biopsy. Clinical follow-up data were available for 160 patients, of which 64 (40%) biopsy results were EXT1/EXT2-positive and 96 (60%) were EXT1/EXT2-negative. The proportion of patients with class 3/4 lupus nephritis coexisting with membranous lupus nephritis was not different between the EXT1/EXT2-positive and EXT1/EXT2-negative groups (25.0% versus 32.3%; P=0.32). The patients who were EXT1/EXT2-negative evolved to ESKD faster and more frequently compared with EXT1/EXT2-positive patients (18.8% versus 3.1%; P=0.003).ConclusionsThe prevalence of EXT1/EXT2 positivity was 32.6% in our cohort of membranous lupus nephritis. Compared with EXT1/EXT2-negative membranous lupus nephritis, EXT1/EXT2-positive disease appears to represent a subgroup with favorable kidney biopsy findings with respect to chronicity indices. Cases of membranous lupus nephritis that are EXT1/EXT2-negative are more likely to progress to ESKD compared with those that are EXT1/EXT2-positive.

IgA nephropathy: analysis of progression in pediatric patients

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

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

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

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

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

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