IC-MPGN is an ultra-rare, fast-progressing complement-mediated kidney disease characterized by immunoglobulin deposits in the kidneys, which may be idiopathic (primary) or secondary to chronic infections, autoimmune disorders, or monoclonal gammopathies. The clinical presentation and disease course are comparable to complement 3 glomerulopathy, also characterized by membranoproliferative histology. Dysregulation of the alternative pathway (AP) is strongly implicated in the pathogenesis of both glomerulonephritis entities including children and adults.
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Webb et al. (2024) studied this question.
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