Correlation of Immune Markers With Outcomes in Biliary Atresia Following Intravenous Immunoglobulin Therapy

• DOI: 10.1002/hep4.1332
• Publication Date: 2019-03-25T14:51:59Z
• Authors: Sehee Kim; Jeffrey Moore; Estella Alonso; Joseph Bednarek; Jorge A. Bezerra; Catherine Goodhue; Saul J. Karpen; Kathleen M. Loomes; John C. Magee; Vicky L. Ng; Averell H. Sherker; Caroline Smith; Cathie Spino; Veena Venkat; Kasper Wang; Ronald J. Sokol; Cara L. Mack
• Keywords: Immunophenotyping

Biliary atresia is a progressive fibroinflammatory cholangiopathy of infancy that associated with activation innate and adaptive immune responses targeting bile ducts. A recently completed multicenter phase I/IIA trial intravenous immunoglobulin in biliary did not improve serum total bilirubin levels at 90 days after hepatoportoenterostomy or survival the native liver 1 year. mechanistic aim this was to determine if peripheral blood immunophenotype clinical outcomes. Flow cytometry cell markers (natural killer [NK], macrophage subsets, T‐ B‐cell regulatory T cells), neutrophils, (clusters differentiation [CD]38, CD69, CD86, human leukocyte antigen‐DR isotype [HLA‐DR]) performed on 29 patients baseline 60, 90, 180, 360 hepatoportoenterostomy. Plasma cytokines neutrophil products were also measured. Spearman correlations change an marker from day revealed increase correlated 1) increased percentage HLA‐DR + CD38 NK cells expression CD69 HLA‐DR, 2) decreased cells, 3) interleukin (IL)‐8 (elastase extracellular traps). Cox modeling 60 plasma IL‐8 risk transplant death by 360. Conclusion: Poor outcomes higher lower cells. Future studies should include immunotherapies these pathways order protect tree ongoing damage.