Open AccessFirst case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia
Author(s) -
Hironori Masutani,
Kosuke Okuwaki,
Mitsuhiro Kida,
Hiroshi Yamauchi,
Hiroshi Inoue,
Shiro Miyazawa,
Tomohisa Iwai,
Miyoko Takezawa,
Wasaburo Koizumi
Publication year - 2014
Publication title -
world journal of gastroenterology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.427
H-Index - 155
eISSN - 2219-2840
pISSN - 1007-9327
DOI - 10.3748/wjg.v20.i26.8740
Subject(s) - autoimmune pancreatitis , medicine , autoimmune hemolytic anemia , primary sclerosing cholangitis , autoimmune hepatitis , gastroenterology , anemia , pancreatitis , pathology , hepatitis , disease
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.