Case Report
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World J Gastroenterol. Jul 14, 2014; 20(26): 8740-8744
Published online Jul 14, 2014. doi: 10.3748/wjg.v20.i26.8740
First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia
Hironori Masutani, Kosuke Okuwaki, Mitsuhiro Kida, Hiroshi Yamauchi, Hiroshi Imaizumi, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Wasaburo Koizumi
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
Author contributions: Masutani H and Okuwaki K contributed equally to this work; Masutani H, Okuwaki K, and Yamauchi H performed the research; Kida M, Miyazawa S, Iwai T, Takezawa M and Imaizumi H contributed to technical support; Kida M, Imaizumi H and Koizumi W contributed to critical revision of the manuscript for important intellectual content; Masutani H wrote the paper.
Correspondence to: Hironori Masutani, MD, Department of Gastroenterology, Kitasato University East Hospital, 2-1-1 Asamizodai, Minami-ku, Sagamihara, Kanagawa 252-0380, Japan. masutani@kitasato-u.ac.jp
Telephone: +81-42-7489111 Fax: +81-42-7498690
Received: January 21, 2014
Revised: March 1, 2014
Accepted: April 5, 2014
Published online: July 14, 2014
Processing time: 173 Days and 20.1 Hours
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.

Keywords: IgG4-related sclerosing cholangitis; Immunoglobulin G4-related sclerosing cholangitis; Autoimmune hemolytic anemia; Autoimmune hemolytic anemia; Autoimmune pancreatitis

Core tip: 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.