As for innate immune response, Watanabe et al. reported that activation of toll-like receptors (TLRs) and nucleotide-binding oligomerization domain- (NOD-) like receptors (NLRs) in monocytes and basophils of patients with IgG4 related disease (IgG4-RD) induced IgG4 production by B cells via B cell activating factor (BAFF) [33, 34]. Here, TNFSF13B is linked to immunoglobulin G4-related sclerosing disease.