Anti-factor B antibodies in atypical hemolytic uremic syndrome.
In: Pediatric Nephrology, Jg. 39 (2024-06-01), Heft 6, S. 1909-1916
Online
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Zugriff:
Background: The etiology of atypical hemolytic uremic syndrome (aHUS) is unknown in 30–40% of patients. Anti-factor B (FB) antibodies are reported in C3 glomerulopathy (C3G) and immune-complex membranoproliferative glomerulonephritis (IC-MPGN), though not in aHUS. Methods: We screened patients < 18-year-old from cohorts of aHUS and C3G/idiopathic IC-MPGN. Anti-FB IgG antibodies were measured by ELISA and confirmed by Western blot. Normative levels were based on antibody levels in 103 healthy blood donors. Results: Prevalence of anti-FB antibodies was 9.7% (95% CI 6.1–14.5%; n = 21) in 216 patients with aHUS, including 11.5% (95% CI 6.4–18.5%; n = 14) in anti-FH associated aHUS and 11.8% (95% CI 4.4–23.9%; n = 6) in patients without a definitive genetic or autoimmune etiology. Patients with significant genetic variants did not show anti-FB antibodies. In patients with concomitant anti-FB and anti-FH antibodies, median anti-FH titers were higher (11,312 AU/mL vs. 4920 AU/mL; P = 0.04). Anti-FB antibody titer correlated with disease severity (hemoglobin and platelets; P < 0.05), declined following plasma exchange and increased during relapse. While 4/64 patients with C3G (6.3%) and 1/17 with IC-MPGN showed anti-FB antibodies, titers were higher in aHUS (544.8 AU/mL vs. 1028.8 AU/mL; P = 0.003). Conclusion: Anti-FB antibodies are present in 6–10% of patients with aHUS and C3G/IC-MPGN, with higher titers in the former. The diagnostic and therapeutic implication of anti-FB antibodies in aHUS needs confirmation and further studies. The study shows propensity for autoantibody generation and co-existence of multiple risk factors for aHUS in Indian children. [ABSTRACT FROM AUTHOR]
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Anti-factor B antibodies in atypical hemolytic uremic syndrome.
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Autor/in / Beteiligte Person: | Khandelwal, Priyanka ; Nambiar, Shreesha ; Saini, Rahul ; Saini, Savita ; Coshic, Poonam ; Sinha, Aditi ; Hari, Pankaj ; Palanichamy, Jayanth Kumar ; Bagga, Arvind |
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Zeitschrift: | Pediatric Nephrology, Jg. 39 (2024-06-01), Heft 6, S. 1909-1916 |
Veröffentlichung: | 2024 |
Medientyp: | academicJournal |
ISSN: | 0931-041X (print) |
DOI: | 10.1007/s00467-024-06284-x |
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