ArticlePloS one2013
Treatment response in Kawasaki disease is associated with sialylation levels of endogenous but not therapeutic intravenous immunoglobulin G.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers, 1 of them a synthesis that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
32 citing papers in PubMed, 1 synthesis or guideline pooled it, 62 citations in OpenAlex.
- The prognostic role of abnormal liver function in IVIG unresponsiveness in Kawasaki disease: a meta-analysis.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2016Pooled it
- Differences in GlycA and lipoprotein particle parameters may help distinguish acute kawasaki disease from other febrile illnesses in children.BMC pediatrics · 2016Trial
- Trial
- Research on gamma globulin unresponsive Kawasaki disease: A review.World journal of clinical pediatrics · 2025Review
- Article
- Charting a course for global progress in PIDs by 2030 - proceedings from the IPOPI global multi-stakeholders' summit (September 2023).Frontiers in immunology · 2024Article
- Glycosylation shapes the efficacy and safety of diverse protein, gene and cell therapies.Biotechnology advances · 2023Review
- Article
- Exploration of Potential Biomarker Genes and Pathways in Kawasaki Disease: An IntegratedFrontiers in genetics · 2022Article
- IgG subclass and Fc glycosylation shifts are linked to the transition from pre- to inflammatory autoimmune conditions.Frontiers in immunology · 2022Review
- A functional spleen contributes to afucosylated IgG in humans.Scientific reports · 2021Article
- Identification of novel locus associated with coronary artery aneurysms and validation of loci for susceptibility to Kawasaki disease.European journal of human genetics : EJHG · 2021Article
- Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development.International journal of molecular sciences · 2021Review
- Kawasaki Disease Patient Stratification and Pathway Analysis Based on Host Transcriptomic and Proteomic Profiles.International journal of molecular sciences · 2021Article
- Aberrantly glycosylated IgG elicits pathogenic signaling in podocytes and signifies lupus nephritis.JCI insight · 2021Article
- Sialylated immunoglobulin G: a promising diagnostic and therapeutic strategy for autoimmune diseases.Theranostics · 2021Review
- N-glycosylated IgG in patients with kidney transplants increases calcium/calmodulin kinase IV in podocytes and causes injury.American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons · 2021Article
- Immunoglobulin G Glycosylation in Diseases.Experientia supplementum (2012) · 2021Article
- Sialylated Immunoglobulins for the Treatment of Immuno-Inflammatory Diseases.International journal of molecular sciences · 2020Review
- Engineering of Fc Multimers as a Protein Therapy for Autoimmune Disease.Frontiers in immunology · 2020Review
Corrections and comments
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Authors and funding
13 authors at 5 institutions in 2 countries.
Funding
Abstract
objectivesAlthough intravenous immunoglobulin (IVIG) is highly effective in Kawasaki disease (KD), mechanisms are not understood and 10-20% of patients are treatment-resistant, manifesting a higher rate of coronary artery aneurysms. Murine models suggest that α2-6-linked sialic acid (α2-6Sia) content of IVIG is critical for suppressing inflammation. However, pro-inflammatory states also up-regulate endogenous levels of β-galactoside:α2-6 sialyltransferase-I (ST6Gal-I), the enzyme that catalyzes addition of α2-6Sias to N-glycans. We asked whether IVIG failures correlated with levels of α2-6Sia on infused IVIG or on the patient's own endogenous IgG.
methodsWe quantified levels of α2-6Sia in infused IVIG and endogenous IgG from 10 IVIG-responsive and 10 resistant KD subjects using multiple approaches. Transcript levels of ST6GAL1, in patient whole blood and B cell lines were evaluated by RT-PCR. Plasma soluble (s)ST6Gal-I levels were measured by ELISA.
resultsThere was no consistent difference in median sialylation levels of infused IVIG between groups. However, α2-6Sia levels in endogenous IgG, ST6GAL1 transcript levels, and ST6Gal-I protein in serum from IVIG-resistant KD subjects were lower than in responsive subjects at both pre-treatment and one-year time points (p <0.001, respectively).
conclusionsOur data indicate sialylation levels of therapeutic IVIG are unrelated to treatment response in KD. Rather, lower sialylation of endogenous IgG and lower blood levels of ST6GALI mRNA and ST6Gal-I enzyme predict therapy resistance. These differences were stable over time, suggesting a genetic basis. Because IVIG-resistance increases risk of coronary artery aneurysms, our findings have important implications for the identification and treatment of such individuals.
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