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2016 ; 31
(5
): 717-25
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Update on C3 glomerulopathy
#MMPMID25326473
Barbour TD
; Ruseva MM
; Pickering MC
Nephrol Dial Transplant
2016[May]; 31
(5
): 717-25
PMID25326473
show ga
C3 glomerulopathy refers to a disease process in which abnormal control of
complement activation, degradation or deposition results in predominant C3
fragment deposition within the glomerulus and glomerular damage. Recent studies
have improved our understanding of its pathogenesis. The key abnormality is
uncontrolled C3b amplification in the circulation and/or along the glomerular
basement membrane. Family studies in which disease segregates with structurally
abnormal complement factor H-related (CFHR) proteins demonstrate that abnormal
CFHR proteins are important in some types of C3 glomerulopathy. This is currently
thought to be due to the ability of these proteins to antagonize the major
negative regulator of C3 activation, complement factor H (CFH), a process termed
'CFH de-regulation'. Recent clinicopathological cohort studies have led to
further refinements in case definition, culminating in a 2013 consensus report,
which provides recommendations regarding investigation and treatment. Early
clinical experience with complement-targeted therapeutics, notably C5 inhibitors,
has also now been published. Here, we summarize the latest developments in C3
glomerulopathy.
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