Clinical Features of IgG4-related Sclerosing Cholangitis: A Study of 36 Cases

  Objective  To summarize the clinical features of IgG4-related sclerosing cholangitis (IgG4-SC).

  Methods  Clinical data of 36 cases with IgG4-SC hospitalized in Peking Union Medical College Hospital in the period of January 2004 to December 2012 were retrospectively analyzed, including symptoms, laboratory tests, imaging results, pathological results and follow-up records. All the cases were diagnosed according to 2012 Japanese clinical diagnostic criteria of IgG4-SC.

  Results  The sex ratio of the 36 IgG4-SC patients was 0.24:1. The mean age of onset was (62.8±9.2) years. The most common symptoms were jaundice (77.8%, 28/36) and abdominal pain (50.0%, 18/36). Serum bilirubin level was normal in 8 cases (22.2%), among whom serum gamma-glutamyl transpeptidase (GGT) level was also normal in 3 cases. Endoscopic ultrasound found bile duct wall thickening in 34 cases (94.4%), significantly more sensitive than abdominal ultrasound (8.3%, 3/36) and CT scan (33.3%, 12/36) (P < 0.05). Seven patients underwent brush cytology or biopsy of bile duct, and the pathological results were all negative. Relapses occurred in 39.1%(9/23) of the 23 cases who were followed up for over 2 years. The patients with more extrabiliary organs or more biliary segments involved were more prone to relapse.

  Conclusions  IgG4-SC is primarily found in middle-aged and elderly men, with clinical manifestations of chronic cholangitis. However, some patients might have no signs of biliary obstruction. The sensitivity of biliary biopsy is poor. The radiological uniform bile duct wall thickening in non-stricture segments is highly suggestive of IgG4-SC. Endoscopic ultrasound should be used as a routine test in suspected cases. Close follow-up is necessary for IgG4-SC patients to detect relapse or concurrent malignancy.