Although these patients were in preliminary disease stages and tested negative for anti-Ro/SSA and anti-La/SSB they displayed increased markers of the canonic TLR9 pathway, compatible with previous reports [31]: IL-1β was elevated in SjS gland ducts (p = 0.05), Fig. 6A) and NFκB (P65) was increased in both acini and ducts (p = 0.0002) (Fig. 6B). Here, NFKB1 is linked to Schwartz-Jampel syndrome.