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

Autori
Garam N, Prohászka Z, Szilágyi Á, Aigner C, Schmidt A, Gaggl M, Sunder-Plassmann G, Bajcsi D, Brunner J, Dumfarth A, Cejka D, Flaschberger S, Flögelova H, Haris Á, Hartmann Á, Heilos A, Mueller T, Rusai K, Arbeiter K, Hofer J, Jakab D, Sinkó M, Szigeti E, Bereczki C, Janko V, Kelen K, Reusz GS, Szabó AJ, Klenk N, Kóbor K, Kojc N, Knechtelsdorfer M, Laganovic M, Lungu AC, Meglic A, Rus R, Kersnik-Levart T, Macioniene E, Miglinas M, Pawłowska A, Stompór T, Podracka L, Rudnicki M, Mayer G, Romana Rysava, Reiterova J, Saraga M, Tomáš Seeman, Zieg J, Sládková E, Szabó T, Capitanescu A, Stancu S, Tisljar, Miroslav ; Galesic, Kresimir ; Tislér A, Vainumäe I, Windpessl M, Zaoral T, Zlatanova G, Csuka D.

Izvornik
Orphanet Journal of Rare Diseases (1750-1172) 8 (2019), 14; 247-261

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
C4 nephritic factor, C3 glomerulopathy, Membranoproliferative glomerulonephritis, C3 nephritic factor, Dense deposit disease, C3 glomerulonephritis

Sažetak
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.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA