International Urology and Nephrology, 2025 (SCI-Expanded, Scopus)
C3 glomerulopathy (C3G) is a rare kidney disease characterized by dysregulation of the alternative pathway of the complement system. Efficacy of the available treatment options is quite limited, and almost half of the patients progress to kidney failure within 10 years. Moreover, the disease is known to recur in 42–100% of patients after kidney transplantation. Conventional treatment approaches typically include a renin angiotensin system blocker and a combination of glucocorticoids and mycophenolic acid derivatives. Successful use of eculizumab has been reported in aggressive disease forms. Response to these strategies have been quite variable, and efficacy of eculizumab has not been shown in slowly progressive forms of C3G. New agents such as iptacopan and pegcetacoplan look very promising. Notably, use of both agents was recently approved by the United States Food and Drug Administration. Herein we review novel treatment strategies for patients suffering from C3G with a focus on agents targeting complement system.