Clinical diagnostic criteria of IgG4-related sclerosing cholangitis
Diffuse or segmental narrowing of the intrahepatic and/or extrahepatic bile duct associated with thickening of bile duct wall on biliary tract imaging
Elevated serum IgG4 levels (≥ 135 mg/dL)
Coexistence of autoimmune pancreatitis, IgG4-related dacryoadenitis/sialadenitis, or IgG4-related retroperitoneal fibrosis
Marked lymphocytic and plasma cell infiltration and fibrosis
Infiltration of IgG4-positive plasma cells (> 10 IgG4-positive plasma cells/hpf)
Storiform fibrosis
Obliterative phlebitis
Effectiveness of corticosteroid therapy
Criteria not met
Reference
Hirotaka Ohara, Kazuichi Okazaki, Hirohito Tsubouchi et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci. 2012 Sep;19(5):536-42.
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