Abstract
Membranoproliferative glomerulonephritis (MPGN) and C3 glomerulopathy are characterised by inflammation with deposition of immunoproteins (i.e. immunoglobulins and/or complement proteins) in the glomerulus and manifest clinically with proteinuria, haematuria and often progressive renal impairment. It is now recognised that the disorders usually arise as a result of pathological immune system activation – either as a result of chronic infectious or autoimmune diseases or else due to complement alternative pathway activation, which can be due to specific autoantibodies called nephritic factors or, less commonly, monogenic disorders. Unfortunately, no specific therapy has been shown to be beneficial in these conditions so current treatment aims to suppress the underlying cause of disease and to provide renoprotective therapy, for instance, using renin-angiotensin system blockade. Because they result from systemic disorders, disease recurrence following transplantation is likely.
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Notes
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Complement haemolytic activity: patient serum across a range of dilutions is used to lyse antibody-coated sheep erythrocytes. Lack of haemolysis at a given dilution suggests deficiency of complement component(s).
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Goodship THJ, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference. Kidney Int. 2017;91(3):539–51.
Sethi S, Fervenza FC. Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification. Semin Nephrol. 2011;31(4):341–8.
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Gale, D., Owen-Casey, M. (2022). Membranoproliferative Glomerulonephritis and C3 Glomerulopathy. In: Harber, M. (eds) Primer on Nephrology. Springer, Cham. https://doi.org/10.1007/978-3-030-76419-7_23
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