In this descriptive case series, trends toward improved renal function and hematuria at 3 months were observed in patients with severe RPGN who received eculizumab in addition to standard immunosuppression, with earlier initiation and milder baseline renal impairment appearing to correlate with more favorable outcomes.
In AAV patients, mean serum creatinine significantly decreased from baseline to 3 months following eculizumab plus standard immunosuppressive therapy.
In AAV patients, mean urinary red blood cell count significantly decreased from baseline to 3 months.
In anti-GBM disease patients, mean serum creatinine significantly decreased from baseline to 3 months.
In anti-GBM disease patients, reductions in uRBC and UPCR at 3 months did not reach statistical significance.
Four of six dialysis-dependent patients at baseline became dialysis-independent after treatment.
All anti-GBM antibodies and nearly all MPO-ANCA seroconverted to negative at 3 months.
Eculizumab was associated with a limited adverse event profile over the follow-up period.
Earlier initiation of eculizumab and milder baseline renal impairment appeared to correlate with more favorable outcomes.
The authors concluded that definitive evidence on the efficacy and safety of eculizumab in RPGN must await larger, prospective, controlled investigations.
This research describes the outcomes of 12 patients with a severe, rapidly worsening form of kidney disease called rapidly progressive glomerulonephritis (RPGN), which was caused either by anti-GBM disease or a condition called ANCA-associated vasculitis (AAV). These patients received a drug called eculizumab—which works by blocking a part of the immune system called the complement pathway—on top of their usual treatments. After three months, kidney function improved significantly in both groups, with blood creatinine levels (a marker of kidney health) falling meaningfully. Additionally, six patients who initially required dialysis were able to stop dialysis after treatment, and harmful antibodies in the blood largely disappeared. Side effects were mild, with only one COVID-19 infection and one oral ulcer recorded across all patients. This research suggests that adding eculizumab to standard treatment may help improve kidney function and reduce inflammation in severe cases of these rare kidney diseases, and that starting treatment earlier with less severe kidney damage at the outset may lead to better results. However, because this was a very small case series of only 12 patients with no comparison group and considerable differences between patients, it is impossible to determine whether eculizumab itself caused these improvements or whether they resulted from the standard treatments already being given. The authors are careful to frame these findings only as real-world observations that raise questions worth investigating further, rather than as proof that eculizumab works. Larger clinical trials with control groups will be needed before any firm conclusions about this drug's effectiveness in these conditions can be drawn.
Li X, Zhang Y, Ke B. (2026). Eculizumab in rapidly progressive glomerulonephritis: a case-series report.. Frontiers in immunology. https://doi.org/10.3389/fimmu.2026.1919699