Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis

Gastrointest Endosc. 2004 Dec;60(6):937-44. doi: 10.1016/s0016-5107(04)02229-1.


Background: Sclerosing cholangitis with autoimmune pancreatitis has a cholangiographic appearance that is similar to that of primary sclerosing cholangitis, but only the former responds well to corticosteroid therapy. It, therefore, is necessary to distinguish between these two diseases. Cholangiography is the reference standard for the diagnosis of primary sclerosing cholangitis. The present study compared the characteristic findings for these two types of sclerosing cholangitis.

Methods: Cholangiograms from patients with primary sclerosing cholangitis (n = 29) and sclerosing cholangitis with autoimmune pancreatitis (n = 26) were studied with regard to length and region of stricture formation, and other characteristic findings.

Results: Band-like stricture, beaded or pruned-tree appearance, and diverticulum-like formation were significantly more frequent in primary sclerosing cholangitis. In contrast, segmental stricture, long stricture with prestenotic dilatation and stricture of the distal common bile duct were significantly more common in sclerosing cholangitis with autoimmune pancreatitis. Discriminant analysis based on these findings correctly identified 27 of 28 patients with primary sclerosing cholangitis and 25 of 26 patients with sclerosing cholangitis with autoimmune pancreatitis. It also identified a patient with an incorrect diagnosis of primary sclerosing cholangitis who proved, on review of a surgical specimen, to have findings consistent with lymphoplasmacytic sclerosing cholangitis.

Conclusions: Characteristic cholangiographic features allow discrimination of sclerosing cholangitis with autoimmune pancreatitis and lymphoplasmacytic sclerosing cholangitis without pancreatitis from primary sclerosing cholangitis.

MeSH terms

  • Adult
  • Aged
  • Autoimmune Diseases / diagnostic imaging*
  • Autoimmune Diseases / pathology
  • Bile Ducts, Extrahepatic / pathology
  • Cholangiopancreatography, Endoscopic Retrograde*
  • Cholangitis, Sclerosing / diagnostic imaging*
  • Cholangitis, Sclerosing / pathology
  • Choledochal Cyst / diagnostic imaging
  • Choledochal Cyst / pathology
  • Cholestasis, Extrahepatic / diagnostic imaging
  • Cholestasis, Extrahepatic / pathology
  • Common Bile Duct / pathology
  • Diagnosis, Differential
  • Female
  • Humans
  • Lymphocytes / pathology
  • Male
  • Middle Aged
  • Pancreatitis / diagnostic imaging*
  • Pancreatitis / pathology
  • Plasma Cells / pathology
  • Sensitivity and Specificity