title: Differentiating immunoglobulin g4-related sclerosing cholangitis from hilar cholangiocarcinoma.
abstract: OBJECTIVE
  Few studies have differentiated immunoglobulin G (IgG) 4-related sclerosing cholangitis (IgG4-SC) from hilar cholangiocarcinoma (CC). Thus, we sought to investigate useful features for differentiating IgG4-SC from hilar CC.
  
  
  METHODS
  We retrospectively compared clinical, serological, imaging, and histological features of six patients with IgG4-SC and 42 patients with hilar CC.
  
  
  RESULTS
  In patients with hilar CC, obstructive jaundice was more frequent (p<0.01), serum total bilirubin levels were significantly higher (p<0.05), serum CA19-9 levels were significantly higher (p<0.01), and serum duke pancreatic monoclonal antigen type 2 levels were frequently elevated (p<0.05). However, in patients with IgG4-SC, the serum IgG (p<0.05) and IgG4 (p<0.01) levels were significantly higher and frequently elevated. The pancreas was enlarged in all IgG4-SC patients but only in 17% of hilar CC patients (p<0.01). Salivary and/or lacrimal gland swelling was detected in only 50% of IgG4-SC patients (p<0.01). Endoscopic retrograde cholangiography revealed that the hilar or hepatic duct was completely obstructed in 83% of hilar CC patients (p<0.01). Lower bile duct stenosis, apart from hilar bile duct stenosis, was more frequent in IgG4-SC patients (p<0.01). Bile duct wall thickening in areas without stenosis was more frequent in IgG4-SC patients (p<0.01).
  
  
  CONCLUSIONS
  An integrated diagnostic approach based on clinical, serological, imaging, and histological findings is necessary to differentiate IgG4-SC from hilar CC.
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
authors.forename: Taku
authors.initials: T
authors.lastname: Tabata
authors.forename: Terumi
authors.initials: T
authors.lastname: Kamisawa
authors.forename: Seiichi
authors.initials: S
authors.lastname: Hara
authors.forename: Sawako
authors.initials: S
authors.lastname: Kuruma
authors.forename: Kazuro
authors.initials: K
authors.lastname: Chiba
authors.forename: Go
authors.initials: G
authors.lastname: Kuwata
authors.forename: Takashi
authors.initials: T
authors.lastname: Fujiwara
authors.forename: Hideto
authors.initials: H
authors.lastname: Egashira
authors.forename: Koichi
authors.initials: K
authors.lastname: Koizumi
authors.forename: Junko
authors.initials: J
authors.lastname: Fujiwara
authors.forename: Takeo
authors.initials: T
authors.lastname: Arakawa
authors.forename: Kumiko
authors.initials: K
authors.lastname: Momma
authors.forename: Masanao
authors.initials: M
authors.lastname: Kurata
authors.forename: Goro
authors.initials: G
authors.lastname: Honda
authors.forename: Koji
authors.initials: K
authors.lastname: Tsuruta
authors.forename: Takao
authors.initials: T
authors.lastname: Itoi
keywords: Endoscopic retrograde cholangiopancreatography; Hilar cholangiocarcinoma; IgG4-ralated sclerosing cholangitis; Immunoglobulin G
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