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recent treatment for IgA Nephropathy

Yuko Shima, Koichi Nakanishi, Masashi Sato, Taketsugu Hama, Hironobu Mukaiyama, Hiroko Togawa, Ryojiro Tanaka, Kandai Nozu, Mayumi Sako, Kazumoto Iijima, Hiroyuki Suzuki, Norishige Yoshikawa
BACKGROUND: Despite a low incidence, nephrotic syndrome (NS) can present with IgA nephropathy (IgAN). The clinical characteristics and long-term outcomes of pediatric patients with IgAN presenting with NS (NS-IgAN) at onset have not been fully elucidated. METHODS: We retrospectively analyzed 426 patients, and compared clinical and pathological (Oxford) findings between those with NS-IgAN and those with non-NS-IgAN. RESULTS: Among 426 patients, 30 (7...
October 6, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Rosanna Coppo
IgA nephropathy (IgAN) is a common chronic glomerular disease that, in most patients, slowly progresses to ESRD. The immune and autoimmune responses that characterize IgAN indicate a potential benefit for corticosteroids. The 2012 Kidney Disease Improving Global Outcome (KDIGO) guidelines suggest giving corticosteroids to patients with rather preserved renal function (GFR>50 ml/min per 1.73 m(2)) and persistent proteinuria >1 g/d, despite 3-6 months of optimized supportive care with renin-angiotensin system blockers...
September 26, 2016: Journal of the American Society of Nephrology: JASN
Tomohiro Terasaka, Haruhito A Uchida, Ryoko Umebayashi, Keiko Tsukamoto, Keiko Tanaka, Masashi Kitagawa, Hitoshi Sugiyama, Hiroaki Tanioka, Jun Wada
BACKGROUND: A link between IgA nephropathy and Crohn's disease has recently been reported. Other researchers hypothesize that intestine-derived IgA complexes deposit in glomerular mesangial cells, eliciting IgA nephropathy. Intestinal mucosal plasma cells mainly secrete IgA2. Nevertheless, IgA1 deposition is strongly implicated as being the primary cause of IgA nephropathy. CASE PRESENTATION: A 46-year-old Japanese man developed IgA nephropathy 29 years ago, following tonsillectomy...
September 5, 2016: BMC Nephrology
Na Liu, Yingfeng Shi, Shougang Zhuang
BACKGROUND: Autophagy is the degrading process of protein and organelles mediated by lysosomes. This process is involved in purging senescent organelles and subversive proteins while maintaining the stability of the intracellular environment. This phenomenon is highly conservative, existing in nearly every species, and is involved in cell growth, proliferation and tumorigenesis. SUMMARY: In recent decades, with the discovery of autophagy-related genes and proteins in conjunction with the improvement in detection methods, the study of autophagy is constantly achieving new breakthroughs...
April 2016: Kidney Diseases
Richard J Glassock
A Polar Views discussion by Pozzi and Rauen et al. on the interpretation and clinical application of the recently published Supportive Versus Immunosuppressive Therapy of Progressive IgA Nephropathy (STOP-IgAN) trial has elucidated important points concerning potential strengths and weaknesses of this landmark randomized trial. This critical examination of the impact of steroid monotherapy or steroid plus an immunosuppressive (IS) agent compared with 'supportive' therapy with inhibitors of the renin-angiotensin system (RAS) has enhanced our appreciation of the importance of rigorous application of titrated RAS inhibition in high-risk patients with persistent proteinuria >0...
August 11, 2016: Nephrology, Dialysis, Transplantation
Thomas Rauen, Frank Eitner, Christina Fitzner, Jürgen Floege, Claudio Pozzi
A comprehensive supportive therapy approach constitutes the mainstay treatment of IgA nephropathy (IgAN) patients. In our recent Supportive versus immunosuppressive Therapy Of Progressive IgA Nephropathy (STOP-IgAN) trial, we systematically selected for patients at high risk of a progressive disease course and evaluated the effect of immunosuppression, combined with supportive care, on renal end points in these patients. There was a higher rate of full clinical remission and transient proteinuria reduction in immunosuppressed patients...
August 11, 2016: Nephrology, Dialysis, Transplantation
Michio Fukuda, Osamu Hotta, Masashi Mizuno, Yoshiaki Ogiyama, Nobuyuki Ohte
Proportions of elderly aged ≥65 and ≥75 within Japan will increase to 30 and 20 %, respectively, in 2025, when "Baby-Boom Generations" will reach the age of 75 years. Okabayashi and colleagues report that even in elderly patients with IgA nephropathy (IgAN), immunosuppressive treatment can reduce proteinuria, with no adverse events. Their findings remind us of recent finding from STOP-IgAN study; additional immunosuppressive therapy to intensive supportive care [specifically renin-angiotensin system (RAS) inhibitors (RASi)] did not improve the outcome...
July 21, 2016: Clinical and Experimental Nephrology
Dita Maixnerova, Colin Reily, Qi Bian, Michaela Neprasova, Jan Novak, Vladimir Tesar
We have summarized the latest findings on markers for progression of immunoglobulin A (IgA) nephropathy (IgAN), the most common primary glomerulonephritis with a high prevalence among end-stage renal disease (ESRD) patients. The clinical predictors of renal outcome in IgAN nephropathy, such as proteinuria, hypertension, and decreased estimated glomerular filtration rate (eGFR) at the time of the diagnosis, are well known. The Oxford classification of IgAN identified four types of histological lesions (known as the MEST score) associated with the development of ESRD and/or a 50 % reduction in eGFR...
August 2016: Journal of Nephrology
Seung Seok Han, Seung Hee Yang, Murim Choi, Hang-Rae Kim, Kwangsoo Kim, Sangmoon Lee, Kyung Chul Moon, Joo Young Kim, Hajeong Lee, Jung Pyo Lee, Ji Yong Jung, Sejoong Kim, Kwon Wook Joo, Chun Soo Lim, Shin-Wook Kang, Yon Su Kim, Dong Ki Kim
TNF superfamily member 13 (TNFSF13) has been identified as a susceptibility gene for IgA nephropathy in recent genetic studies. However, the role of TNFSF13 in the progression of IgA nephropathy remains unresolved. We evaluated two genetic polymorphisms (rs11552708 and rs3803800) and plasma levels of TNFSF13 in 637 patients with IgA nephropathy, and determined the risk of ESRD according to theses variable. Neither of the examined genetic polymorphisms associated with a clinical outcome of IgA nephropathy. However, high plasma levels of TNFSF13 increased the risk of ESRD...
April 11, 2016: Journal of the American Society of Nephrology: JASN
Jürgen Floege, Thomas Rauen
The optimal role of immunosuppressive therapy in the treatment of IgA nephropathy is controversial. Results of a recently completed randomized controlled trial provide strong support for comprehensive supportive care rather than immunosuppressive therapy in patients at high risk for progression.
January 2016: Kidney International
Claudio Pozzi, Cristina Sarcina, Francesca Ferrario
IgA Nephropathy leads young people to dialysis more often than other glomerular diseases, because often diagnosis and therapy are made late. Nephrologists waive to treat IgAN pts with chronic renal insufficiency, believing that treatment may not be effective and safe. Moreover, studies in IgAN pts with reduced renal function are lacking. Small studies seem to indicate a possible utility of RAS blockers and corticosteroids in these patients. Recently, VALIGA study showed that corticosteroids and immunosuppressants were more frequently used in pts with eGFR <30 ml/min than in those with eGFR >30 ml/min (60 vs...
August 2016: Journal of Nephrology
Claudio Pozzi
The therapy of IgA nephropathy (IgAN) is cause for debate among nephrologists. Since the early 1980s, many therapeutic attempts have been proposed, but most of them did not prove efficacy. The recent KDIGO Clinical Practice Guideline for Glomerulonephritis recommend long-term ACE-I or ARB treatment when proteinuria is more than 1 g/day, with up-titration of the drug. For patients with GFR >50 ml/min and proteinuria persistently higher than 1 g/day, they suggest a 6-month course of corticosteroid therapy...
February 2016: Journal of Nephrology
Liyu He, Xiaofei Peng, Guoyong Liu, Chengyuan Tang, Hong Liu, Fuyou Liu, He Zhou, Youming Peng
IgA nephropathy (IgAN) is the finding of immune deposits predominantly containing polymeric IgA in the glomerular mesangium on renal biopsy. Recently studies show that inflammation may involve in the progression of renal glomerulosclerosis and tubulointerstitial scarring in IgAN. This study was designed to evaluate the renoprotective effect of triptolide on IgAN rat model. IgAN was induced in Sprague-Dawley rats by oral and intravenous immunization with BSA for 12 weeks. Rats were treated with triptolide (200 μg/kg/d intragastrically) from 12 to 28 weeks...
2015: Immunopharmacology and Immunotoxicology
Riccardo Magistroni, Vivette D D'Agati, Gerald B Appel, Krzysztof Kiryluk
Recent years have brought notable progress in the field of IgA nephropathy. Here, we highlight important new directions and latest developments, including successful discovery of several genetic susceptibility loci, formulation of the multihit pathogenesis model, introduction of the Oxford pathology scoring system, and formalization of the Kidney Disease Improving Global Outcomes (KDIGO) consensus treatment guidelines. We focus on the latest genetic findings that confirm a strong contribution of inherited factors and explain some of the geoethnic disparities in disease susceptibility...
November 2015: Kidney International
E Mamatov, A F Kocaay, M A Koc, Z K Celebi, S Sengül, K Keven, H Tutkak, A Tuzuner
BACKGROUND: Renal transplantation is the best choice for the treatment of dialysis patients with end-stage renal failure because it provides better quality of life and more life time. However, despite successful surgical techniques, immunological issues in kidney transplantation are not completely resolved. Thus, after transplantation, patients must be followed up closely. Although patient follow-up with the use of creatinine and renal biopsy are common, it is thought that biopsy is too invasive and that creatinine is unreliable...
July 2015: Transplantation Proceedings
See Cheng Yeo, Adrian Liew, Jonathan Barratt
Despite advances in our understanding of immunoglobulin A nephropathy (IgAN) over the past decade, there are currently no specific therapies capable of targeting key pathways involved in the pathogenesis of the disease. Recent studies have, however, provided new insights into important molecular pathways that are likely to be amenable to therapeutic manipulation in the future. Specifically, a deeper understanding of the role of mucosal immunity, B-cell activation and mesangial cell activation in IgAN has provided the impetus for a number of exciting phase II/III clinical trials in IgAN...
November 2015: Nephrology
Edgard Wehbe, Charbel Salem, James F Simon, Sankar D Navaneethan, Marc Pohl
Background and objectives. The mesangial deposition of IgA is rarely described with proliferative glomerulonephritis associated with Staphylococcus infection. Recently, this association has been increasingly recognized possibly due to the increased rate of Staphylococcus infection. Design setting, participants and measurements. We report two cases of methicillin-sensitive Staphylococcus aureus bacteremia associated with acute proliferative glomerulonephritis with dominant mesangial deposit of IgA. We searched MEDLINE (1960-2009) for similar reports...
June 2011: NDT Plus
Huijun Shen, Weizhong Gu, Jianhua Mao, Xiujuan Zhu, Xia Jin, Haidong Fu, Aimin Liu, Qiang Shu, Lizhong Du
BACKGROUND: IgA nephropathy (IgAN) is the most common primary glomerulonephritis in children and adolescents worldwide. Meanwhile, minimal change disease (MCD) is the most common cause of nephrotic syndrome in children. Recently, several case reports have revealed that IgAN can be complicated by MCD in adults. Here, we report our experience concerning the features of such patients in pediatrics. METHODS: The clinical manifestations and pathological features of 197 children who presented with IgAN from December 2007 to November 2013 were analyzed retrospectively based on the criteria for nephrotic syndrome and MCD...
2015: Nephron
Francesco P Schena, Fabio Sallustio, Grazia Serino
miRNAs are regulators of gene expression in diverse biological and pathological courses in life. Their discovery may be considered one of the most important steps in the story of modern biology. miRNAs are packed within exosomes and released by cells for cellular communications; they are present in bodily fluids. Their study opens the way for understanding the pathogenetic mechanisms of many diseases; furthermore, as potential candidate biomarkers, they can be measured in bodily fluids for non-invasive monitoring of disease outcomes...
June 2015: Clinical Science (1979-)
Samih H Nasr, Ahmet Dogan, Christopher P Larsen
Amyloidosis derived from leukocyte cell-derived chemotaxin 2 is a recently recognized form of amyloidosis, and it has already been established as a frequent form of systemic amyloidosis in the United States, with predominant involvement of kidney and liver. The disease has a strong ethnic bias, affecting mainly Hispanics (particularly Mexicans). Additional ethnic groups prone to develop amyloidosis derived from leukocyte cell-derived chemotaxin 2 include Punjabis, First Nations people in British Columbia, and Native Americans...
November 6, 2015: Clinical Journal of the American Society of Nephrology: CJASN
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