C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy /

Nóra Garam; Zoltán Prohászka; Ágnes Szilágyi; Christof Aigner; Alice Schmidt; Martina Gaggl; Gere Sunder-Plassmann; Dóra Bajcsi; Jürgen Brunner; Alexandra Dumfarth; Daniel Cejka; Stefan Flaschberger; Hana Flögelova; Ágnes Haris; Ágnes Hartmann; Andreas Heilos; Thomas Mueller; Krisztina Rusai; Klaus Arbeiter; Johannes Hofer; Dániel Jakab; Mária Sinkó; Erika Szigeti; Csaba Bereczki; Viktor Janko; Kata Kelen; György S Reusz; Attila J Szabó; Nóra Klenk; Krisztina Kóbor; Nika Kojc; Maarten Knechtelsdorfer; Mario Laganovic; Adrian Catalin Lungu; Anamarija Meglic; Rina Rus; Tanja Kersnik-Levart; Ernesta Mačionienė; Marius Miglinas; Anna Pawłowska; Tomasz Stompór; Ludmila Podracka; Michael Rudnicki; Gert Mayer; Romana Rysava; Jana Reiterova; Marijan Saraga; Tomáš Seeman; Jakub Zieg; Eva Sládková; Tamás Szabó; Andrei Capitanescu; Simona Stancu; Miroslav Tisljar; Kresimir Galesic; András Tislér; Inga Vainumäe; Martin Windpessl; Tomas Zaoral; Galia Zlatanova; Dorottya Csuka

doi:10.1186/s13023-019-1237-8

Title	C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy /
Authors	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 ; Mačionienė, Ernesta ; Miglinas, Marius ; Pawłowska, Anna ; Stompór, Tomasz ; Podracka, Ludmila ; Rudnicki, Michael ; Mayer, Gert ; Rysava, Romana ; Reiterova, Jana ; Saraga, Marijan ; Seeman, Tomáš ; 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
DOI	10.1186/s13023-019-1237-8
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Is Part of	Orphanet journal of rare diseases.. London : BioMed Central Ltd.. 2019, vol. 14, art. no. 247, p. [1-14].. eISSN 1750-1172
Keywords [eng]	C3 glomerulonephritis ; C3 glomerulopathy ; C3 nephritic factor ; C4 nephritic factor ; Dense deposit disease ; Membranoproliferative glomerulonephritis
Abstract [eng]	Background: Acquired or genetic abnormalities of the complement alternative pathway are the primary cause of C3glomerulopathy(C3G) but may occur in immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) as well. Less is known about the presence and role of C4nephritic factor(C4NeF) which may stabilize the classical pathway C3-convertase. Our aim was to examine the presence of C4NeF and its connection with clinical features and with other pathogenic factors. 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.
Published	London : BioMed Central Ltd
Type	Journal article
Language	English
Publication date	2019
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„C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy /“