Efficacy of iptacopan in idiopathic immune complex-mediated membranoproliferative glomerulonephritis refractory to conventional treatment - PubMed
7 days ago
- #iptacopan
- #MPGN-IC
- #complement-inhibitors
- Idiopathic immune complex-mediated membranoproliferative glomerulonephritis (MPGN-IC) is diagnosed after excluding common causes like infections, autoimmune diseases, and gammopathies.
- MPGN-IC involves activation of the alternative complement pathway, often indicated by low serum C3 levels.
- No specific treatment exists for MPGN-IC, but immunosuppressive agents (e.g., prednisone, mycophenolate, cyclophosphamide, rituximab) are used based on severity.
- A case of aggressive idiopathic MPGN-IC with nephritic-nephrotic syndrome and rapid renal deterioration unresponsive to conventional therapy was treated with iptacopan, an oral factor B inhibitor of the alternative complement pathway.
- Complete remission was achieved after 12 months of iptacopan treatment, highlighting its potential efficacy in refractory cases.