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E-Poster Submission Deadline
Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
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Abstract titles should be brief and reflect the content of the abstract.
C3G is a complex, ultra rare, progressive primary glomerulonephritis with an estimated annual incidence of 1-2 cases per million. The pathogenesis of C3G centers on the overactivation of the alternative complement pathway (AP) as a result of nephritic factors or genetic mutations. Until recently there was no approved targeted therapy for C3G and the suggested management options (KDIGO 2021, but not based on controlled studies) include supportive care including blood pressure management-antiproteinuric therapy with an angiotensin-converting enzyme inhibitor or angiotensin II receptor blocker, immunosuppression and life-style modifications. The efficacy of these measures is limited. There is a high unmet medical need for new therapies to treat C3G.. Iptacopan, which has been recently approved by US FDA and EMA for C3G is a proximal complement inhibitor that specifically binds factor B and inhibits the AP. The present clinical case aims to assess the clinical efficacy, safety, tolerability of iptacopan in a patient with C3G for a period of 10 months.
Female patient of 62 years old was presented to her doctor with anemia (Hb 10.5) and peripheral oedema. She was referred for hematologist's clinical and laboratory estimation , which was unremarkable. Then, patient presented to renal clinic with eGFR 32 mils/min and proteinuria 3300 mg/24 hours. C3 complement was below normal range. Νο other issues from the medical history of the patient were revealed. Kidney biospy was performed and the diagnosis was C3G. After exclusion of possible secondary forms of C3G, iptacopan was provided to our patient. Four weeks before starting the treatment with iptacopan the patient received meningococcal, pneumococcal and haemophilus influenzae vaccination.
Within 3 weeks after starting the treatment with iptacopan( 200 mg twice daily), kidney function of the patient improved (e GFR: 54 mils/min), proteinuria significantly decreased to a level of 654.8 mg/ 24 hours and C3 complement levers returned to normal range. To date, the treatment with iptacopan is continued and is well tolerated with no adverse side effects and it is worth to mention that the patient is under the monotherapy of iptacopan for 10 months. Gradually proteinuria is within normal range (0.08grs/ 24 hours) and renal function remains stable.
Our case report opens an interesting therapeutic field for the use of complement blockers for the treatment of C3G. From this point-of view, the current case report , shows that iptacopan is highly effective regarding the reduction of proteinuria and recovery and stabilization of kidney function and is well tolerated. Our case report suggests that even under the 10-month monotherapy, iptacopan may become a promising oral treatment option for C3G.
