Changes in βGL1-22/LGL1–specific T cells in GD patients correlates with disease severity. (A) Percentages of CD1d⁻ βGL1-22–, LGL1-, or α-GalCer–specific T cells were compared between healthy controls and GD patients. (B) Graph shows the correlation between percentage of CD1d-βGL1-22 tetrameter-positive cells, LGL1 tetrameter-positive cells, or type I NKT cells and chitotriosidase activity in type 1 GD patients. (C) Representative dot plot representing the changes in the percentage of LGL1-specific T cells in GD patients before and after treatment (top). Lower panel shows graph summarizing changes in the percentage of LGL1-specific T cells in GD patients before and after treatment (n = 4) (bottom). (D) Multiplex cytokine analysis of sera from GD patients (n = 29) and healthy subjects (n = 15). Analytes with significant differences compared with healthy control sera are shown. Data are shown as mean + SEM. ***P < .0001; **P < .001; *P < .01. MIP-1β, macrophage inflammatory protein 1β; sIL-2Rα, soluble IL-2 receptor α.