IgG4-related disease presenting with acute kidney injury and tubulointerstitial nephritis

Immunoglobulin G-4 related disease (IgG4-RD) is an immune-mediated, inflammatory disease that often involves multiple organ systems. IgG4-RD can be classified according to the organs involved. Type 1 IgG4-RD is related to acute pancreatitis and sclerosing cholangitis. Disease manifestation is also seen in the retroperitoneal region, pelvic organs, and orbital space. Here we describe a rare case of IgG4-RD causing isolated acute kidney injury.