title: First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia.
abstract: To our knowledge, patients with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) associated with autoimmune hemolytic anemia (AIHA) have not been reported previously. Many patients with IgG4-SC have autoimmune pancreatitis (AIP) and respond to steroid treatment. However, isolated cases of IgG4-SC are difficult to diagnose. We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis. The patient was a 73-year-old man who was being treated for dementia. Liver dysfunction was diagnosed on blood tests at another hospital. Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis, but a rapidly progressing anemia developed simultaneously. After the diagnosis of AIHA, steroid treatment was begun, and the biliary stricture improved. IgG4-SC without AIP was thus diagnosed.
authors.affiliation: Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi, Department of Gastroenterology, Kitasato University East Hospital, Kanagawa 252-0380, Japan.
authors.forename: Hironori
authors.initials: H
authors.lastname: Masutani
authors.affiliation: Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi, Department of Gastroenterology, Kitasato University East Hospital, Kanagawa 252-0380, Japan.
authors.forename: Kosuke
authors.initials: K
authors.lastname: Okuwaki
authors.affiliation: Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi, Department of Gastroenterology, Kitasato University East Hospital, Kanagawa 252-0380, Japan.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kida
authors.affiliation: Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi, Department of Gastroenterology, Kitasato University East Hospital, Kanagawa 252-0380, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yamauchi
authors.affiliation: Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi, Department of Gastroenterology, Kitasato University East Hospital, Kanagawa 252-0380, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Imaizumi
authors.affiliation: Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi, Department of Gastroenterology, Kitasato University East Hospital, Kanagawa 252-0380, Japan.
authors.forename: Shiro
authors.initials: S
authors.lastname: Miyazawa
authors.affiliation: Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi, Department of Gastroenterology, Kitasato University East Hospital, Kanagawa 252-0380, Japan.
authors.forename: Tomohisa
authors.initials: T
authors.lastname: Iwai
authors.affiliation: Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi, Department of Gastroenterology, Kitasato University East Hospital, Kanagawa 252-0380, Japan.
authors.forename: Miyoko
authors.initials: M
authors.lastname: Takezawa
authors.affiliation: Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi, Department of Gastroenterology, Kitasato University East Hospital, Kanagawa 252-0380, Japan.
authors.forename: Wasaburo
authors.initials: W
authors.lastname: Koizumi
mesh_terms: D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; D007074:Immunoglobulin G; D011239:Prednisolone
keywords: Autoimmune hemolytic anemia; Autoimmune pancreatitis; IgG4-related sclerosing cholangitis; Immunoglobulin G4-related sclerosing cholangitis