Hypergammaglobulinemia and the existence of disease-related autoantibodies (for example, those against lactoferrin [15], carbonic anhydrase II (CAII) [16], amylase-alpha 2A [17], pancreatic secretory trypsin inhibitor (PSTI) [18], and plasminogen-binding protein peptide [19]) support the hypothesis that autoimmunity may participate in the pathogenesis of IgG4-RD. The gene discussed is SPINK1; the disease is immunoglobulin G4-related sclerosing disease.