Complement dysregulation leads to C3 overactivation.1–4
The loss of regulation results in excessive accumulation of C3 in the kidney.
In primary IC-MPGN, in addition to C3 deposition, Ig deposits are a hallmark of disease, and a primary driver of classical pathway activation.1–4, 8–10
C3 dysregulation is central to the pathogenesis of C3G and primary IC-MPGN and leads to renal inflammation and damage.6,11,12
Aspaveli is the first and only approved targeted C3 and C3b inhibitor that acts on the 3 key steps of C3 dysregulation, by:4,5
Blocking C3 cleavage by all C3 convertases4,5
Blocking the AP C3 convertase, thereby inhibiting the amplification loop5,13
Blocking C3b, leading to reduced glomerular C3 deposition and generation of downstream effectors of kidney inflammation and damage4,5
Downloadable document
EMA indication:
ASPAVELI® is indicated for the treatment of adult and adolescent patients aged 12 to 17 years with C3 glomerulopathy (C3G) or primary immune-complex membranoproliferative glomerulonephritis (IC-MPGN) in combination with a renin-angiotensin system (RAS) inhibitor, unless RAS inhibitor treatment is not tolerated or contraindicated.5
| For the full EMA Aspaveli SmPC, please click here |
▼This medicine is subject to additional monitoring. This will allow quick identification of new safety information. Healthcare professionals are asked to report any suspected adverse reactions via their national pharmacovigilance reporting system. Suspected adverse reactions should also be reported to Sobi via email at [email protected].
Abbreviations
C3, complement 3; C3G, C3 glomerulopathy; IC-MPGN, immune complex-mediated membranoproliferative glomerulonephritis; RAS, renin-angiotensin system.
References
Halfon M, et al. Kidney Int Rep 2024;10(1):75-86.
Meuleman MS, et al. Semin Immunol 2022;60:101634.
Heidenreich K, et al. Front Nephrol 2024;4:1460146.
Bomback AS, et al. Kidney Int Rep 2025;10:17–28.
Aspaveli EMA Summary of product characteristics. Swedish Orphan Biovitrum AB (publ) January 2026.
Smith RJH, et al. Nat Rev Nephrol 2019;15:129–43.
Bomback AS, et al. Kidney Int Rep 2024;10(1)17–28
Cook HT and Pickering MC. Nat Rev Nephrol 2015;11(1):14–22.
Donadelli R et al. Front Immunol 2018; 15:9:2329.
Kovala M et al. Front Immunol 2018;15:9:2329.
Dunkelberger JR, Song WC. Cell Res 2010;20(1):34–50.
Schena FP, et al. Int J Mol Sci. 2020;21(2):525.
Zwarthoff SA, et al. Front Immunol 2018:9:1691.