Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team) and the other from lymph proliferation (Umehara team) supported by the “Research Program for Intractable Disease” of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as “IgG4-RD” and proposed the comprehensive diagnostic criteria (CDC) for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP), compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease) and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity. 1. Introduction Recent studies have suggested simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis, which lead us to propose the concept of a systemic disease [1, 4, 10, 23, 24]. However, there are many synonyms suggesting a systemic disease, such as IgG4-related autoimmune disease [1], IgG4-related sclerosing disease [4], IgG4-related plasmacytic syndrome (SIPS) [23], IgG4-related multiorgan lymphoproliferative syndrome (IgG4-MOLPS) [10], and systemic IgG4-related disease, all of which may refer to the same conditions [24, 25] (Table 1). To simplify these conditions, members of two Japanese research committees for IgG4-related disease, one from view of fibrosclerosis (Chaired by Prof. Okazaki) [24] and the other from lymph proliferation (Chaired by Professor. Umehara H) [25], both of which are supported by the “Research for Intractable Disease” Program from the Ministry of Health, Labor, and Welfare of Japan, have agreed with unification of different nomenclatures as “IgG4-related disease (IgG4-RD)” and proposed the comprehensive diagnostic criteria (CDC) for IgG4-RD [15]. As it still remains unclear whether pathogenetic mechanisms in each involved organ-are same or not, the term IgG4-RD was appointed as minimally reflecting these conditions to avoid misdiagnosis of malignancy as much as possible. Table 1:
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