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Edgar Allan Poe/Sullivan's Island Library
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West Ashley Library
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Wando Mount Pleasant Library
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Village Library
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Anti-factor B antibodies in atypical hemolytic uremic syndrome.
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![loading](/sites/all/modules/hf_eds/images/loading.gif)
- Author(s): Khandelwal, Priyanka; Nambiar, Shreesha; Saini, Rahul; Saini, Savita; Coshic, Poonam; Sinha, Aditi; Hari, Pankaj; Palanichamy, Jayanth Kumar; Bagga, Arvind
- Source:
Pediatric Nephrology. Jun2024, Vol. 39 Issue 6, p1909-1916. 8p. - Source:
- Additional Information
- Subject Terms: AUTOANTIBODY analysis; IMMUNOGLOBULIN analysis; RISK assessment; RESEARCH funding; ENZYME-linked immunosorbent assay; HEMOGLOBINS; HEMOLYTIC-uremic syndrome; COMPLEMENT (Immunology); SEVERITY of illness index; DESCRIPTIVE statistics; GLOMERULONEPHRITIS; GENETIC variation; VOLUMETRIC analysis; BLOOD platelets; WESTERN immunoblotting; CONFIDENCE intervals; DISEASE relapse; GENETICS
- Subject Terms:
- Abstract: 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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