Among SjS patient cell subsets, monocytes secrete increased levels of pro-inflammatory cytokines such as interleukin(IL)-6 and B cell-activating factor (BAFF) upon stimulation [7], express type I interferon (IFN)-regulated genes [5, 8–10], reduced NFκB inhibitor (IκBα) [11], and show decreased function in phagocytosis of apoptotic cells [12]. This evidence concerns the gene NFKBIA and Schwartz-Jampel syndrome.