Promotion of B cell activation, plasmacyte differentiation, and germinal center formation by BAFF and APRIL [42], and ectopic germinal center formation in lacrimal and salivary glands from patients with MD [43] suggest that inappropriate BAFF and APRIL may contribute to progressive plasmacyte infiltration and ectopic germinal center formation in the target organs of patients with IgG4-RD. The gene discussed is TNFSF13; the disease is Menkes disease.