title: Hepatobiliary IgG4 Cholangiopathy: Case Series and Literature Review.
abstract: IgG4 cholangiopathy is a recently described distinctive type of hepatobiliary manifestation of IgG4-related disease. This is often misdiagnosed as a malignancy of the hepatobiliary system on clinical presentation and imaging. Accurate diagnosis is crucial for appropriate management. Histopathology provides crucial information and is characterized by IgG4-positive lymphoplasmacytic infiltration with storiform fibrosis and obliterative phlebitis. We present the clinicopathological spectrum of a retrospective series of 6 cases of IgG4 cholangiopathy that clinically masqueraded as hepatobiliary malignancies.
authors.affiliation: Department of Pathology, Institute of Liver & Biliary Sciences, New Delhi, India drarchanarastogi@gmail.com.
authors.forename: Archana
authors.initials: A
authors.lastname: Rastogi
authors.affiliation: Department of Pathology, Institute of Liver & Biliary Sciences, New Delhi, India.
authors.forename: Chhagan
authors.initials: C
authors.lastname: Bihari
authors.affiliation: Department of Pathology, Institute of Liver & Biliary Sciences, New Delhi, India.
authors.forename: Shrruti
authors.initials: S
authors.lastname: Grover
authors.affiliation: Department of Pathology, Institute of Liver & Biliary Sciences, New Delhi, India.
authors.forename: Apurba
authors.initials: A
authors.lastname: Rajbongshi
authors.affiliation: Department of HPB Surgery, Institute of Liver & Biliary Sciences, New Delhi, India.
authors.forename: Asit
authors.initials: A
authors.lastname: Arora
authors.affiliation: Department of HPB Surgery, Institute of Liver & Biliary Sciences, New Delhi, India.
authors.forename: N
authors.initials: N
authors.lastname: Nikhil
authors.affiliation: Department of HPB Surgery, Institute of Liver & Biliary Sciences, New Delhi, India.
authors.forename: Viniyendra
authors.initials: V
authors.lastname: Pamecha
authors.affiliation: Department of Hepatology, Institute of Liver & Biliary Sciences, New Delhi, India.
authors.forename: Shiv Kumar
authors.initials: SK
authors.lastname: Sarin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology*; D001649:Bile Duct Diseases / Q000276:immunology / Q000473:pathology*; D005260:Female; D005355:Fibrosis / Q000276:immunology / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G; D008107:Liver Diseases / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology*
chemical_list: D007074:Immunoglobulin G
keywords: IgG4; cholangiopathy; hepatobiliary; obliterative phlebitis; storiform fibrosis