IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder recognized as a novel clinical entity with either synchronous or metachronous multiorgan involvement.
Autoimmune pancreatitis (AIP) is classified into two types:
type 1 AIP as a pancreatic manifestation of
IgG4-RD and
type 2 AIP with granulocytic epithelial lesion and occasional association with
ulcerative colitis. Although the pathogenic mechanism still remains unclear, possible multipathogenic factors such as genetic factors, disease-specific or related
antigens, and abnormal innate or adaptive immunity may be involved in the development of
IgG4-RD. Many immunocytes including M2 macrophages, plasmablasts, B cells, and T-cells (Th2-CD4+T, follicular helper T-cells, and CD4+SLAMF7+cytotoxic T-cells) play important roles in the pathogenesis. Conventional induction and maintenance
therapies with
glucocorticoid or
rituximab are recommended in all symptomatic patients with active
IgG4-RD. In those at risk for irreversible damage in any organs, this should be done urgently, regardless of symptoms. As no randomized clinical trials other than
glucocorticoid maintenance
therapy for
type 1 AIP have been performed, the comprehensive management for
IgG4-RD has not been established yet. Targeted treatment approaches against the plasmablast to B cell lineage and the CD4+ SLAMF7+ cytotoxic T-cell seem to be promising for the future-directed treatment.