“…Therefore, a novel clinicopathological entity, IgG4-related sclerosing disease or IgG4-related systemic disease (IgG4-RSD), was proposed and AIP was determined to be a subset of IgG4-RSD that involved pancreatic lesions (5, 6). IgG4-RSD has been identified in nearly every organ system, including the liver, gallbladder and organs outside the gastrointestinal tract such as the salivary glands, lachrymal glands, breasts, lungs, adenohypophysis, retroperitoneum, lymph nodes and aorta (2,7,8). Although there are accumulating numbers of cases of IgG4-RSD, the number of patients with IgG4-related nephropathy is comparatively small.…”