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      C3-glomerulonephritis in New Zealand – a case series

      1 , , 2

      BMC Nephrology

      BioMed Central

      C3 glomerulonephritis, New Zealand, Chronic kidney disease

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          Abstract

          Background

          C3-glomerulonephritis can lead to progressive renal impairment from complement-mediated glomerular injury. Incidence and outcomes of C3-glomerulonephritis are not known in the New Zealand population.

          Methods

          We reviewed all cases of C3-glomerulonephritis from the past 10 years at a tertiary referral centre in New Zealand. Descriptive information on baseline characteristics and clinical outcomes was collected.

          Results

          Twenty-six patients were included (16 men; mean ± SD age 44 ± 25 years) with a median follow-up of 30 months. Disease incidence was 1.3 cases per million individuals, of which 42% were Pacific Islanders. Most patients presented with renal impairment, with a median (IQR) creatinine at diagnosis of 210 (146–300) μmol/L, and 11 (42%) patients presented with nephrotic syndrome. Seven (27%) patients progressed to end stage renal disease and 2 (8%) had died. End stage renal disease occurred in 20% of patients treated with immunosuppression and in 50% of those not treated. Complete remission was seen in 25% of patients treated with some form of immunosuppression and in 17% of those not treated.

          Conclusions

          Our results are consistent with previous descriptions of C3-glomerulonephritis. There was a suggestion of better clinical outcomes in patients treated with immunosuppression. There was a higher disease incidence in Pacific Islanders, which may indicate an underlying susceptibility to complement dysfunction in this population.

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          Most cited references 17

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          C3 glomerulopathy: consensus report

          C3 glomerulopathy is a recently introduced pathological entity whose original definition was glomerular pathology characterized by C3 accumulation with absent or scanty immunoglobulin deposition. In August 2012, an invited group of experts (comprising the authors of this document) in renal pathology, nephrology, complement biology, and complement therapeutics met to discuss C3 glomerulopathy in the first C3 Glomerulopathy Meeting. The objectives were to reach a consensus on: the definition of C3 glomerulopathy, appropriate complement investigations that should be performed in these patients, and how complement therapeutics should be explored in the condition. This meeting report represents the current consensus view of the group.
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            Eculizumab for dense deposit disease and C3 glomerulonephritis.

            The principle defect in dense deposit disease and C3 glomerulonephritis is hyperactivity of the alternative complement pathway. Eculizumab, a monoclonal antibody that binds to C5 to prevent formation of the membrane attack complex, may prove beneficial. In this open-label, proof of concept efficacy and safety study, six subjects with dense deposit disease or C3 glomerulonephritis were treated with eculizumab every other week for 1 year. All had proteinuria >1 g/d and/or AKI at enrollment. Subjects underwent biopsy before enrollment and repeat biopsy at the 1-year mark. The subjects included three patients with dense deposit disease (including one patient with recurrent dense deposit disease in allograft) and three patients with C3 glomerulonephritis (including two patients with recurrent C3 glomerulonephritis in allograft). Genetic and complement function testing revealed a mutation in CFH and MCP in one subject each, C3 nephritic factor in three subjects, and elevated levels of serum membrane attack complex in three subjects. After 12 months, two subjects showed significantly reduced serum creatinine, one subject achieved marked reduction in proteinuria, and one subject had stable laboratory parameters but histopathologic improvements. Elevated serum membrane attack complex levels normalized on therapy and paralleled improvements in creatinine and proteinuria. Clinical and histopathologic data suggest a response to eculizumab in some but not all subjects with dense deposit disease and C3 glomerulonephritis. Elevation of serum membrane attack complex before treatment may predict response. Additional research is needed to define the subgroup of dense deposit disease/C3 glomerulonephritis patients in whom eculizumab therapy can be considered.
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              New approaches to the treatment of dense deposit disease.

              The development of clinical treatment protocols usually relies on evidence-based guidelines that focus on randomized, controlled trials. For rare renal diseases, such stringent requirements can represent a significant challenge. Dense deposit disease (DDD; also known as membranoproliferative glomerulonephritis type II) is a prototypical rare disease. It affects only two to three people per million and leads to renal failure within 10 yr in 50% of affected children. On the basis of pathophysiology, this article presents a diagnostic and treatment algorithm for patients with DDD. Diagnostic tests should assess the alternative pathway of complement for abnormalities. Treatment options include aggressive BP control and reduction of proteinuria, and on the basis of pathophysiology, animal data, and human studies, plasma infusion or exchange, rituximab, sulodexide, and eculizumab are additional options. Criteria for treatment success should be prevention of progression as determined by maintenance or improvement in renal function. A secondary criterion should be normalization of activity levels of the alternative complement pathway as measured by C3/C3d ratios and C3NeF levels. Outcomes should be reported to a central repository that is now accessible to all clinicians. As the understanding of DDD increases, novel therapies should be integrated into existing protocols for DDD and evaluated using an open-label Bayesian study design.
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                Author and article information

                Contributors
                hari.talreja@middlemore.co.nz
                Journal
                BMC Nephrol
                BMC Nephrol
                BMC Nephrology
                BioMed Central (London )
                1471-2369
                17 September 2020
                17 September 2020
                2020
                : 21
                Affiliations
                [1 ]GRID grid.414055.1, ISNI 0000 0000 9027 2851, Auckland City Hospital, ; Auckland, New Zealand
                [2 ]GRID grid.415534.2, ISNI 0000 0004 0372 0644, Department of Renal Medicine, , Middlemore Hospital, ; 100 Hospital Rd, Otahuhu, Auckland, 2025 New Zealand
                Article
                2056
                10.1186/s12882-020-02056-5
                7495885
                © The Author(s) 2020

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                Categories
                Research Article
                Custom metadata
                © The Author(s) 2020

                Nephrology

                chronic kidney disease, new zealand, c3 glomerulonephritis

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