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Recommendations for the management of patients with immune-mediated kidney disease during the severe acute respiratory syndrome coronavirus 2 pandemic
Nephrology Dialysis Transplantation, May 2020 – Watch the video here
Is there long-term value of pathology scoring in immunoglobulin A nephropathy? A validation study of the Oxford Classification for IgA Nephropathy (VALIGA) update
Nephrology Dialysis Transplantation, November 2018
Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients
Kidney International, September 2017, Volume 92, Issue 3
Oxford Classification of IgA nephropathy 2016: an update from IgA Nephropathy Classification Working Group
Kidney International, May 2017, Volume 91, Issue 5
A Multicenter Sudy of the Predictive Value of Crescents in IgA Nephropathy
JASN February 2017
Risk factors for progression in children and young adults with IgA nephropathy: an analysis of 261 cases from the VALIGA European cohort
Paediatric Nephrology, January 2017, Volume 32, Issue 1
Renal biopsy in patients with diabetes: a pooled meta-analysis of 48 studies
Nephrology Dialysis Transplantion, January 2017
Toward Noninvasive Diagnosis of IgA Nephropathy: A Pilot Urinary Metabolomic and Proteomic Study
Disease Markers, Volume 2016 (2016)
EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis
ARD Online First, published on June 23, 2016 as 10.1136/annrheumdis-2016-209133
Renal Biopsy in 2015 – From Epidemiology to Evidence-Based Indications
Am J Nephrol 2016;43:1-19
Corticosteroids in IgA Nephropathy: A Retrospective Analysis from the VALIGA Study
JASN, September 2015
Validation of the Oxford classification of IgA nephropathy in cohorts with different presentations and treatments
Kidney International, April 2014
The ERA-EDTA database on recurrent glomerulonephritis following renal transplantation
Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis
Nephrology Dialysis Transplantion, January 2014
The Kidney, a Victim and Culprit of Autoimmune and Alloimmune Responses
Nephron Clin Pract 2011;119:c200–c204, August 11, 2011
ERA-EDTA Immunonephrology working group
Nephrology Dialysis Transplantation, February 2010
Glomerular diseases and transplantation: similarities in pathogenetic mechanisms and treatment options
Nephrology Dialysis Transplantion, September 2010
UPDATES FROM RECENT PUBLICATIONS IN THE FIELD
Barratt et al (2019) KI Reports in press, Barbour et al (2019) JAMA Intern Med. 179:942
The PROTECT study is a phase III trial, aimed at 280 patients with IgAN, to compare combined AngII-RA and ET-A inhibitor with single blocking of AngII-R. The paper of Barratt describes how this study will implement surrogate efficacy endpoints. To facilitate risk stratification, 2 full prediction models, combining clinical and biopsy to predict disease progression have been described. This study of Barbour and colleagues made use of 3927 patients from different large multi-ethnic cohorts. This can be an important contribution to future clinical trial design.
Chen et al (2019) CJASN in press, Suzuki et al (2019) KI reports in press
In a large study of 1200 patients with biopsy-proven IgAN, it was shown that plasma levels of gal-deficient IgA1 (gdIgA1)/C3 was associated with the development of CKD (Chen et al). In a smaller study (10 IgAN, 20 IgAV), increased levels of both gdIgA1 as well as IgG autoantibodies against gdIgA1 were observed (Suzuki et al). Interestingly, these altered levels of autoantibodies were also observed in B cell cultures from these patients, and were not observed in patients with IgA-vasculitis without renal involvement.
Liu et al (2019) AJKD 74:15
A double blind phase II study has been performed with 60 patients with IgAN (proteinuria 0,75-3 g/d; EGFR >30ml) randomized for oral hydrocloroquine or placebo. Patients in the treatment arm showed a significant reduction in proteinuria. With the notion that reduction in proteinuria seems to become an accepted surrogate end point, this opens the way for further exploration of this antimalarial drug in IgAN, for which a lot experience already excist in the treatment of SLE.
Jarrick et al (2019) JASN 30:866
In Sweden a nation-wide population based study was performed concerning the mortality in IgAN. It was found that patients with IgAN had a 1.5 fold increased risk for death, both compared to the general population as compared to siblings and spouses. There was no increased mortality preceding the ESRD.
Aringer et al (2019) Arthritis & Rheumatol 71:1400
Recently the 2019 EULAR/ACR classification criteria for SLE have been published. This includes positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains. These weighted criteria will help in a better classification of patients.
Arazi et al (2019) Nat Immunol 20:902, Der et al (2019) Nat Immunol 20:915
To increase mechanistic insights into the pathogenesis of lupus nephritis, two separate studies have focussed on single cell sequencing of renal biopsies of patients with LN. Arazi et al concentrated on the immune landscape and isolated and sequenced infiltrating immune cells from 24 biopsies. They identified 21 subsets of immune cells, signs of local B cell activation and a clear IFN-signature in most of these cells. In parallel they looked at the urine sediment and observed a strong correlation in gene expression in these cells.
Within the same consortium, Der et al performed scSeq on renal cells of 21 biopsies of patients with LN. They identified an IFN signature in tubular cells. In parallel they investigated 17 skin biopsies form the same patients, performed scSeq and observed that also keratinocytes showed such an IFN-signature. Both studies open the way to new monitoring tools in addition to the renal biopsy.
Membranous Nephropathy (MN)
Fervenza et al (2019) NEJM 381:36; Seitz-Polski et al (2019) CJASN 14:1173
In the MENTOR trial, 130 patients with Membranous Nephropathy underwent randomization for either rituximab or cyclosporin. At 12 months, it was shown that rituximab was noninferior to cyclosporine in inducing complete or partial remission of proteinuria and was superior in maintaining proteinuria remission. Treatment with rituximab showed a faster and greater magnitude of decline in autoantibodies to anti-PLA2R.
Since different protocols of rituximab treatment have been used, xx et al compared different regimens of rituximab. Participants of the NICE cohort (infusions of 1-g rituximab at 2-week intervals) were compared with participants from the GEMRITUX study (two infusions of 375 mg/m2 at 1-week interval). At month 6, before any treatment modifications, more remission was found in the NICE cohort, which was accompanied by higher levels of circulating rituximab. It is suggested that the higher dose rituximab protocol is more effective in depleting B-cells and thereby prevent epitope spreading which is associated with remission of MN.
Sethi et al (2019) JASN 30:1123
While anti-PLA2R is an established marker of primary membranous nephropathy (MN), there have up to now been no reports of autoantibodies in secondary forms of MN. Using microdissection and mass spectrometry Sethi and co-works were able to identify Exotosin 1 and 2 as antigens in a substantial subset of secondary MN, especially patients with class 5 lupus nephritis, but also some cases with primary MN associated with autoimmune features. Exotosin 1/2 immune complexes were never found in patients with PLA2R antibodies, and the IgG subclass distribution of the autoantibodies differed. No circulating antibodies where detected.
Colucci et al (2019) Kidney Int 96:971
Colucci and co-workers had made the serendipitous finding that some children with nephrotic syndrome had increased amounts of IgM on the surface of their T cell. When studying this in detail they made a series of interesting observations. Increased T cell IgM was found only on patients with steroid sensitive nephrotic syndrome, and not in those with established genetic causes or forms steroid resistant cases. When following steroid sensitive cases over time they found the amount of T cell IgM to be predictive of relapse. However, Increased T cell IgM was not correlated to total IgM levels or markers of acute inflammation such CRP. Rituximab treatment was associated with a reduction of T cell IgM, while relapse after such treatment was associated with reappearance of IgM on the cells. Biochemical analysis suggests that glycosylation differences might explain the binding of IgM to T cells.
- Prognosis and outcome in patients with primary membranous nephropathy project, submitted by IWG, was selected to be one of the projects to be supported by the ERA-EDTA through the Long Term Fellowship Programme.
- Identification of prognostic factors for patients with ANCA associated vasculitis – This project is performed in collaboration with European Vasculitis Society ‘EUVAS and DESCARTES Working Group of the ERA-EDTA. The project aims at identifying prognostic factors present at time of diagnosis regarding relapses, patient and renal survival, risk for cardiovascular disease and malignancy. Secondary aims include the validation of prognostic factors and the development of a cohort with long term outcomes to permit a genomic approach to prognosis. Integration of predictive factors to develop prognostic tools that might also aid treatment selection.
- European VALIdation Study of the Oxford Classification of IGA Nephropathy (VALIGA) – IgA nephropathy (IgAN), the most common glomerular disease worldwide, is potentially progressive to renal failure. In individual patients its course is unpredictable before development of severe proteinuria, hypertension, reduced glomerular filtration rate and renal fibrosis. There is a need to detect progressive cases in early stages, when a therapeutic intervention is more likely to be effective. A breakthrough report just published from an International Consensus -based on a retrospective analysis of 265 adults and children with IgAN from four continents -focuses on prognostic information provided by renal biopsy. According to this Oxford Classification of IgAN, four pathological features (mesangial hypercellularity, segmental glomerulosclerosis, endocapillary hypercellularity, and tubular atrophy/interstitial fibrosis) predict renal outcome independently from all clinicalindicators at the time of biopsy and during follow up (Kidney International 2009;76:534-45; and Kidney International 2009;76:546-56). The limited number of patients and their heterogeneous origin indicate a need for validation studies involving large cohorts of patients. The proposed study will investigate European patients and the results will be complementary to those from similar studies in North America and Asia, allowing a global perspective on the value of these predictive factors Biopsy-proven IgAN with long follow-up or rapidly progressive course (about 500 cases) will be enrolled by 26 Centers of Nephrology and Renal Pathology from 9 Countries. Renal biopsies will be scored by the local pathologist and centrally reviewed in Oxford. Clinical data at renal biopsy and during the follow-up will be provided by local Nephrologists to the Coordinating Center. Statistical analysis will be performed by Canadian experts. This multicenter, multinational study supported by the ERA-EDTA Working Group of Immunonephrology provides with information beyond the validation of the Oxford classification of IgAN, aiming at detecting for each lesion the most effective treatment and the “point of no return” when no treatment is effective.
- IgA nephropathy in Europe and Japan IWG – Background: In studies published in Japan and in Europe there are usually substantial differences in demographics and clinical features. However, these differences have never addressed in a study using similar method to identify cases. Method: An online survey was created consisting of a large number of questions where data of individual patients could be entered on-line. A call was opened and advertised in Europe through the IWG newsletter and homepage and in a similar fashion in Japan. Nephrologists were encourage to complete data on an unspecified number of consecutive patients with biopsy proven IgAN. Results: Data from more than 400 European IgAN and more than 200 Japanese patients have been entered into the database. Preliminary results were presented at the IWG-JSN business meeting in Budapest. There were not so big differences when it comes to severity (eGFR and U-alb), but there were huge differences regarding sex distribution and with regard to respiratory versus gastrointestinal co-morbidities.
- The acute/subacute nephritic syndrome in Europe and Japan – Background: The epidemiology of glomerulonephritis is difficult to compare between countries due to the huge impact of how biopsy indications are applied. A way to reduce the variability is to limit the comparisons clinical situations where there is less disagreement on whether a biopsy should be performed or not. Method: Renal biopsy registries across Europe was contacted through the IWG newsletter or directly and invited to deliver data if they could provide data on the subset of patients having undergone a biopsy on the indication of acute/subacute nephritic syndrome (ANS). This was defined as the combination of hematuria, proteinuria and a rising creatinine in the absence of known chronic renal failure. Results: Data has now been delivered from Japan, Sweden, Scotland, Croatia and Austria. We are still waiting for data from Norway, Italy and Turkey. A poster has been recently accepted at the 57th ERA-EDTA Virtual Congress. Click here to view the poster.
Athens Winter School in Nephrology – Focus in Glomerular Diseases
February 27-29, 2020 – Athens, Greece
Join the Athens Winter meeting 2020, an educational event organised by the Immunonephrology Working Group (IWG) of the ERA EDTA and Mayo Clinic (Rochester USA) in collaboration with Hellenic Society of Nephrology. The meeting is mainly focused on glomerular diseases but also covers a wide spectrum of topics in the field of nephrology such as kidney transplantation, chronic kidney diseases and the relation between pregnancy and the kidneys. Read more
In 2019 the ERA-EDTA organised the Scientific and Educational Interaction Day (SEID) on October 25-26, 2019 in Vienna (Austria). IWG contributed in the creation of the Scientific Programme with the educational session on ‘Systemic diseases affecting the kidney‘
Extrarenal involvement in SLE and its impact on the outcome of pts with LN
Vladimir Tesar, Prague, Czech Republic
Renal involvement in AAV and its treatment
Annette Bruchfeld, Stockholm, Sweden
Anti-GBM disease with and without lung hemorrhage
Marten Segelmark, Lund, Sweden
How to become your local expert in Nephrogenetics – October 8-9, 2018, Belgrade, Serbia – In collaboration with YNP, WGIKD, ERA-EDTA Ethics Committee and ERKnet
Renal Implications of Advances in Immunology and Inflammation– September 19-20, 2017 Malmo, Sweden – In collaboration with the Nordic Society of Nephrology and Mayo Clinic – click here to view the available educational materials.
Pathogenesis and Treatment of IgA Nephropathy –September 16, 2016 Tours, France – click here to view the available educational materials.