Eculizumab in Pediatric Kidney Disorders: A Review
Journal of Pediatric Nephrology,
Vol. 10 No. 3 (2022),
12 December 2022,
Page 103-111
https://doi.org/10.22037/jpn.v10i3.38928
Eculizumab is a humanized monoclonal antibody targeting the C5 (complement 5) member
of complement proteins and inhibiting its cleavage to C5a and C5b. Eculizumab has been
proven to be effective in a wide array of nephrologic, neurologic, and hematologic pediatric
disorders. Kidney disorders, particularly those with immune-mediated pathomechanism,
are the most common indications of eculizumab, including atypical hemolytic uremic
syndrome (aHUS), hemolytic uremic syndrome (HUS), membranoproliferative
glomerulonephritis (MPGN), immunoglobulin A (IgA) nephropathy, hematopoietic stem
cell transplant-associated thrombotic microangiopathy, and less commonly post-infectious
glomerulonephritis and diffuse proliferative lupus nephritis. In this review, we aimed to
summarize the current evidence on approved and off-label applications of eculizumab and
their specific considerations in pediatric kidney disorders.