Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis
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ORIGINAL ARTICLE
Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis Priyanka Khandelwal 1 & Swati Bhardwaj 1 & Geetika Singh 2 & Aditi Sinha 1 & Pankaj Hari 1 & Arvind Bagga 1 Received: 30 April 2020 / Revised: 13 July 2020 / Accepted: 31 July 2020 # IPNA 2020
Abstract Background Data on therapy and outcome of dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and immunecomplex MPGN (IC-MPGN) in children are limited. Methods In this retrospective single-center study from 2007 to 2019, kidney biopsies were reviewed to include patients aged 10 years at onset (HR = 4.0, P = 0.004), and DDD (HR = 4.2, P = 0.02) were independently associated with adverse outcome; achieving remission was protective (HR = 0.04; P < 0.001). Conclusion Outcome in patients with C3 glomerulopathy and IC-MPGN was unsatisfactory, and only a small proportion of patients achieved complete or partial remission. Patients with DDD were more likely to present with rapidly progressive GN and were at higher risk of adverse outcomes, including kidney failure. Keywords Dense deposit disease . Rapidly progressive glomerulonephritis . Calcineurin inhibitor . Mycophenolate mofetil . Cyclophosphamide . Children
Introduction C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis (IC-MPGN) are rare Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00467-020-04736-8) contains supplementary material, which is available to authorized users. * Pankaj Hari [email protected] 1
Division of Nephrology, ICMR Center for Advanced Research in Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India
2
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India
glomerular diseases with high risk of progressive kidney failure [1, 2]. C3 glomerulopathy is classified as C3 deposition that is ≥ 2 orders of magnitude higher than accompanying immunoglobulin, while IC-MPGN shows predominant IgG staining on immunofluorescence microscopy [3–5]. C3 glomerulopathy is further classified into dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) based on the presence or absence, respectively, of ribbon-like intramembranous electron dense deposits [4, 5]. The pathogenetic process underlying C3 glomerulopathy is dysregulation of the alternative complement pathway [6]. C3 nephritic factor (C3Nef), autoantibodies to factors H (FH), B, and C3b, and variants or copy number variations in genes regulating the alternative complement pathway may be found in patients with C3 glomerulopathy and IC-MPGN [7–9]. Therapy, especially in children, is based on case series and expert opinion and often influenced by the degree of proteinuria or kidney dysfunction
Pediatr Nephrol
[9–13]. Immunosuppression with steroids, mycophenolate mofetil, cyclophosphamide, calcineurin inhibitor, and rituximab has not shown consistent benefit; plasmapheresis has been used a
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