TLR9 and Schwartz-Jampel syndrome: An activated interferon-α (IFN-α) signature pathway is involved in the autoimmune process of SjS, and EBV DNA and RNA have been reported to activate plasmacytoid dendritic cells (pDC) through engagement of Toll-like receptor 9 (TLR-9) and TLR-7, and then increase IFN-α production (95, 96).