MxA levels are linked to signs of active disease and decrease in patients treated with hydroxychloroquine, indicating the potential clinical usefulness of MxA in categorizing patients based on IFN positivity (99, 100).Activation of the IFN-I pathway is observed as one of the vital pathways in the pathophysiology of SjS, and it is particularly pronounced in individuals who have antibodies to SSA/SSB (101, 102). This evidence concerns the gene IFNA1 and Schwartz-Jampel syndrome.