title: Recurrent severe acute hepatitis caused by hypereosinophilic syndrome associated with elevated serum immunoglobulin G4 levels.
abstract: A 46-year-old male was admitted to our hospital with severe acute hepatitis, hypereosinophilia, and serum immunoglobulin G4 (IgG4) elevation. Plasma exchange was performed, and he was treated by prednisolone; however, his hepatitis recurred twice over the following twelve months. Transjuglar liver biopsy was performed at the third onset, which demonstrated extensive hepatocyte necrosis, congestion, and severe eosinophil infiltration. We diagnosed hypereosinophilic syndrome (HES)-related hepatitis. Although no cholangitis was detected by imaging and pathological diagnosis, IgG4-positive cells were detected in the liver and bone marrow. Furthermore, the elevation of serum IgG4 levels was associated with the eosinophil count and his clinical condition. After the addition of azathioprine to his treatment regimen, no reoccurrence was observed. IgG4-positive cells may have contributed to the severity and refractoriness of this recurrent acute HES-related hepatitis.
authors.affiliation: Department of Gastroenterology, Juntendo University School of Medicine, 2-1-1 Hongo Bunkyo-ku, Tokyo, 113-8421, Japan, aoyama@juntendo.ac.jp.
authors.forename: Tomonori
authors.initials: T
authors.lastname: Aoyama
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Matsumoto
authors.forename: Akira
authors.initials: A
authors.lastname: Uchiyama
authors.forename: Kazuyoshi
authors.initials: K
authors.lastname: Kon
authors.forename: Shunhei
authors.initials: S
authors.lastname: Yamashina
authors.forename: Satoko
authors.initials: S
authors.lastname: Suzuki
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ikejima
authors.forename: Takashi
authors.initials: T
authors.lastname: Yao
authors.forename: Ryohei
authors.initials: R
authors.lastname: Kuwatsuru
authors.forename: Sumio
authors.initials: S
authors.lastname: Watanabe
mesh_terms: D000208:Acute Disease; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D006505:Hepatitis / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000097:blood* / Q000150:complications* / Q000628:therapy; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011239:Prednisolone / Q000627:therapeutic use; D012008:Recurrence
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G; D011239:Prednisolone