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Pracoviště: Department of Pediatrics University of Debrecen...

4 záznamů v Medvik Filtry

Článek

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

Garam, Nóra
Autor Garam, Nóra Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Kútvölgyi St 4, Budapest, H-1125, Hungary
Prohászka, Zoltán
Autor Prohászka, Zoltán Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Kútvölgyi St 4, Budapest, H-1125, Hungary. prohaszka.zoltan@med.semmelweis-univ.hu
Szilágyi, Ágnes
Autor Szilágyi, Ágnes Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Kútvölgyi St 4, Budapest, H-1125, Hungary
Aigner, Christof
Autor Aigner, Christof Division of Nephrology and Dialysis, Department of Medicine III, Medical University of Vienna, Vienna, Austria
Schmidt, Alice
Autor Schmidt, Alice Division of Nephrology and Dialysis, Department of Medicine III, Medical University of Vienna, Vienna, Austria
Gaggl, Martina
Autor Gaggl, Martina Division of Nephrology and Dialysis, Department of Medicine III, Medical University of Vienna, Vienna, Austria
Sunder-Plassmann, Gere
Autor Sunder-Plassmann, Gere Division of Nephrology and Dialysis, Department of Medicine III, Medical University of Vienna, Vienna, Austria
Bajcsi, Dóra
Autor Bajcsi, Dóra 1st Department of Internal Medicine, University of Szeged, Szeged, Hungary
Brunner, Jürgen
Autor Brunner, Jürgen Department of Pediatrics, Medical University of Innsbruck, Innsbruck, Austria
Dumfarth, Alexandra
Autor Dumfarth, Alexandra Department of Medicine III: Nephrology, Transplant Medicine and Rheumatology, Geriatric Department, Ordensklinikum Linz - Elisabethinen, Linz, Austria

Orphanet journal of rare diseases. 2019 ; 14 (1) : 247. [pub] 20191108

Orphanet J Rare Dis
ISSN 1750-1172
Medvik
Zdroj

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.