Comparative characteristics of the complement system in patients with C3-glomerulopathy and atypical hemolytic uremic syndrome of chronic course who suffered an acute episode of thrombotic microangiopathy

Autor: Valeriya A. Yurova, Natalia L. Kozlovskaya, Larisa A. Bobrova, Leonid V. Kozlov, Svetlana S. Andina, Kseniya A. Demyanova
Jazyk: ruština
Rok vydání: 2023
Předmět:
Zdroj: Терапевтический архив, Vol 95, Iss 6, Pp 475-480 (2023)
Druh dokumentu: article
ISSN: 0040-3660
2309-5342
00403660
DOI: 10.26442/00403660.2023.06.202269
Popis: Aim. To compare changes in the complement system in C3-glomerulopathy (C3-GP) and atypical hemolytic uremic syndrome (aHUS) after the relief of an acute episode of thrombotic microangiopathy. Materials and methods. The study included 8 patients diagnosed with C3-GP and 8 with aHUS in remission. The blood levels of the complement system components were determined: C3, C4, C3a, C5a, factor H (CFH), factor B (CFB), membrane-attacking complex (MAC), antibodies to C3b (anti-C3b-AT), the level of hemolytic activity (CH50), the content of factor D (CFD) in the urine. Results. C3 and CH50 levels were within the reference range in both groups, however, in the C3-GP group they were at the lower limit, and C3 level was significantly lower than in the aHUS group: 0.56 [0.44; 0.96] vs 1.37 [1.16; 2.52] (p=0.003). CFB increased level was detected in both groups, but in the C3-GP group it was significantly lower than in the aHUS group 275.1 [222.1; 356.6] vs 438.7 [323.3; 449.3] (p=0.010). C3a, C5a and MAC levels were increased in both groups, but the maximum was in the C3-GP group, and the MAC level in the C3-GP group was 2 times higher than that in aHUS, and these differences reached statistical significance 123 5556686 vs 56031294 (p=0.036). CFH and CFD levels was increased in both groups, but their highest values was in the aHUS group. Conclusion. Alternative complement pathway activation signs were present in both groups of patients with complement-mediated nephropathies, regardless the stage of the disease. In C3-GP, alternative complement pathway activation was more pronounced than in aHUS after the relief of an acute episode of thrombotic microangiopathy.
Databáze: Directory of Open Access Journals
Externí odkaz: