title: Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings.
abstract: OBJECTIVE
  To assess the clinical, computed tomography (CT), and pathological findings in patients with lymphoplasmacytic sclerosing cholangitis.
  
  
  METHODS
  Fifteen consecutive patients (four women and 11 men, mean age 71 years) with lymphoplasmacytic sclerosing cholangitis and without the characteristic features of underlying disorders causing benign biliary strictures were retrospectively recruited. Two radiologists evaluated multiphase contrast-enhanced CT images acquired with 0.5 or 1-mm collimation. One pathologist performed all histological examinations, including IgG4 immunostaining.
  
  
  RESULTS
  The intrahepatic biliary ducts showed dilatation in all 15 patients, but only seven presented with jaundice. Although laboratory data were not available in all patients, serum gammaglobulin and IgG levels were elevated in five of six patients and six of eight patients, respectively. Anti-nuclear antibody was detected in three of six patients. The involved biliary ducts showed the following CT findings: involvement of the hilar biliary duct (14/15), a mean wall thickness of 4.9 mm, a smooth margin (10/15), a narrow but visible lumen (6/15), hyper-attenuation during the late arterial phase (9/15), homogeneous hyper-attenuation during the delayed phase (11/11), and no vascular invasion (14/15). Abnormal findings in the pancreas and urinary tract were detected in eight of 15 patients. In 13 patients with adequate specimens, moderate to severe lymphoplasmacytic infiltration associated with dense fibrosis was observed. Infiltration of IgG4-positive plasma cells was moderate or severe in nine patients and minimal or absent in four patients.
  
  
  CONCLUSIONS
  Lymphoplasmacytic sclerosing cholangitis exhibits relatively characteristic clinical and CT findings, although they are not sufficiently specific for differentiation from other biliary diseases.
authors.affiliation: Department of Technical Radiology, Nagoya University School of Health Sciences, Nagoya, Japan. shigekimiyo@luck.ocn.ne.jp
authors.forename: S
authors.initials: S
authors.lastname: Itoh
authors.forename: T
authors.initials: T
authors.lastname: Nagasaka
authors.forename: K
authors.initials: K
authors.lastname: Suzuki
authors.forename: H
authors.initials: H
authors.lastname: Satake
authors.forename: T
authors.initials: T
authors.lastname: Ota
authors.forename: S
authors.initials: S
authors.lastname: Naganawa
mesh_terms: D000368:Aged; D000469:Alkaline Phosphatase / Q000097:blood; D000974:Antibodies, Antinuclear / Q000097:blood; D001653:Bile Ducts, Intrahepatic / Q000000981:diagnostic imaging*; D015209:Cholangitis, Sclerosing / Q000000981:diagnostic imaging* / Q000473:pathology; D003287:Contrast Media; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods; D005719:gamma-Globulins / Q000032:analysis
chemical_list: D000974:Antibodies, Antinuclear; D003287:Contrast Media; D007074:Immunoglobulin G; D005719:gamma-Globulins; D000469:Alkaline Phosphatase