title: Two siblings with type 1 autoimmune pancreatitis.
abstract: Type 1 autoimmune pancreatitis (AIP) is characterized by a high serum IgG4 concentration and is closely associated with the HLA-DRB1(*)04:05-DQB1(*)04:01 haplotype, for which family studies may disclose its immunogenetic significance. In the present study, we encountered two male siblings with type 1 AIP who exhibited diffuse pancreatic swelling with a capsule-like rim and diffuse pancreatic duct stricture. The younger brother also displayed characteristic IgG4-related sialadenitis and retroperitoneal fibrosis. Contrary to our expectations, the siblings showed only normal or slightly elevated values of serum IgG4 and no HLA DRB1(*)04:05-DQB1(*)04:01 haplotype, suggesting that type 1 autoimmune pancreatitis is associated with multiple immunogenetic factors.
authors.affiliation: Department of Gastroenterology, Shinshu University School of Medicine, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Watanabe
authors.forename: Masahiro
authors.initials: M
authors.lastname: Maruyama
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Ito
authors.forename: Keita
authors.initials: K
authors.lastname: Kanai
authors.forename: Takaya
authors.initials: T
authors.lastname: Oguchi
authors.forename: Takashi
authors.initials: T
authors.lastname: Muraki
authors.forename: Hideaki
authors.initials: H
authors.lastname: Hamano
authors.forename: Norikazu
authors.initials: N
authors.lastname: Arakura
authors.forename: Masao
authors.initials: M
authors.lastname: Ota
authors.forename: Shigeyuki
authors.initials: S
authors.lastname: Kawa
mesh_terms: D001327:Autoimmune Diseases / Q000145:classification* / Q000235:genetics* / Q000276:immunology; D059811:HLA-DRB1 Chains / Q000235:genetics / Q000276:immunology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000145:classification* / Q000235:genetics* / Q000276:immunology; D035781:Siblings
chemical_list: D059811:HLA-DRB1 Chains