IgG4-Related Disease in Organs Other than the Hepatobiliary-Pancreatic System.

Immunoglobulin G4-related disease (IgG4-RD) is a new disease entity that was established in this century. It is a systemic and chronic inflammatory disorder. Autoimmune pancreatitis is a representative condition, but there is clearly involvement from other organs, including the kidneys, the retroperitoneal cavity, the lungs, and the thyroid, lacrimal, salivary, pituitary, and prostate glands. To aid in the resolution of the numerous issues related to IgG4-RD, the authors established a registry system of patients in multicenter institutes with IgG4-RD-the SMART registry. Using SMART registry data, they provide an overview of the involvement of organs other than the hepatobiliary-pancreatic system in IgG4-RD and emphasize the importance of consultation and cooperation with other specialists in the diagnosis, treatment, and management of patients with IgG4-RD.