MedPage Today—According to three case reports, dense-deposit kidney disease regressed consistently over time during treatment with a monoclonal antibody to complement protein C5.
The improvement persisted during continuous treatment for up to a year, but interruption of eculizumab after 18 months in one case led to a rapid increase in proteinuria.
Authors of all three case reports characterized eculizumab as a potentially valuable therapeutic option for patients with an incurable condition; their cases were reported as letters appearing March 22 in the New England Journal of Medicine.
Dense-deposit disease, or membranoproliferative glomerulonephritis (MPGN), primarily affects children, often progresses to end-stage kidney disease, and can recur after renal transplantation.
MPGN is associated with dysregulation of the alternative complement pathway, arising from mutations in factor H or activation of autoantibodies. Animal models have shown that uncontrolled activation of C5 plays a major role in pathogenesis.