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AIP 1 is characterized by lymphoplasmacytic sclerosing pancreatitis (LPSP) in histopathological assessment, with storiform fibrosis, IgG4-positive plasma cell infiltrations, and presence of other Ig-G4 RD features, including extraintestitial symptoms ( The history of the discovery of IgG4 RD begun since the first description of the association of the elevated serum concentration of IgG4 with clinical features of autoimmune pancreatitis (AIP), by Hamano et al

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