C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy.
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Garam Nóra, Prohászka Zoltán, Szilágyi Ágnes, Aigner Christof, Schmidt Alice, Gaggl Martina, Sunder-Plassmann Gere, Bajcsi Dóra, Brunner Jürgen, Dumfarth Alexandra, Cejka Daniel, Flaschberger Stefan, Flögelova Hana, Haris Ágnes, Hartmann Ágnes, Heilos Andreas, Mueller Thomas, Rusai Krisztina, Arbeiter Klaus, Hofer Johannes, Jakab Dániel, Sinkó Mária, Szigeti Erika, Bereczki Csaba, Janko Viktor, Kelen Kata, Reusz György S, Szabó Attila J, Klenk Nóra, Kóbor Krisztina, Kojc Nika, Knechtelsdorfer Maarten, Laganovic Mario, Lungu Adrian Catalin, Meglic Anamarija, Rus Rina, Kersnik-Levart Tanja, Macioniene Ernesta, Miglinas Marius, Pawłowska Anna, Stompór Tomasz, Podracka Ludmila, Rudnicki Michael, Mayer Gert, Romana Rysava, Reiterova Jana, Saraga Marijan, Tomáš Seeman, Zieg Jakub, Sládková Eva, Szabó Tamás, Capitanescu Andrei, Stancu Simona, Tisljar Miroslav, Galesic Kresimir, Tislér András, Vainumäe Inga, Windpessl Martin, Zaoral Tomas, Zlatanova Galia, Csuka Dorottya
Аннотация
RESULTS: One hunfe IC-MPGN/C3G patients were enrolled in the study. C4NeF activity was determined by hemolytic assay utilizing sensitized sheep erythrocytes. Seventeen patients were positive for C4NeF with lower prevalence of renal impairment and lower C4d level, and higher C3 nephritic factor (C3NeF) prevalence at time of diagnosis compared to C4NeF negative patients. Patients positive for both C3NeF and C4NeF had the lowest C3 levels and highest terminal pathway activation. End-stage renal disease did not develop in any of the C4NeF positive patients during follow-up period. Positivity to other complement autoantibodies (anti-C1q, anti-C3) was also linked to the presence of nephritic factors. Unsupervised, data-driven cluster analysis identified a group of patients with high prevalence of multiple complement autoantibodies, including C4NeF.
CONCLUSIONS: In conclusion, C4NeF may be a possible cause of complement dysregulation in approximately 10-15% of IC-MPGN/C3G patients.