Elsevier

Gastrointestinal Endoscopy

Volume 60, Issue 6, December 2004, Pages 937-944
Gastrointestinal Endoscopy

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

https://doi.org/10.1016/S0016-5107(04)02229-1Get rights and content

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.

Section snippets

Patients and methods

ERCP was performed in 8407 patients and percutaneous transhepatic biliary drainage (PTBD) in 1421 patients (1989-2003). Of the total, 31 met criteria for PSC.8 Of these 31 patients, two were excluded; one because the quality of the cholangiograms was poor and the other because of the presence of bile duct stones.

There also were 27 patients who met the criteria for AIP proposed by the Japanese Pancreatic Society as follows9: (1) diffuse narrowing of the main pancreatic duct with an irregular

Results

Biliary stenosis was mainly observed in the extrahepatic ducts of patients with SC with AIP. Stenosis was noted in both the intrahepatic and extrahepatic ducts with PSC, except for two cases in which it was present only in the intrahepatic ducts (Table 1). The most prominent cholangiographic feature of SC with AIP was stenosis of the distal common bile duct. However, sclerosing changes in the intrahepatic and extrahepatic bile ducts or stricture in the hilar region also were observed in half of

Discussion

Visualization of the biliary tract is essential for confirmation of the diagnosis of PSC; endoscopic retrograde cholangiography is the method of choice. Adequate cholangiograms also can be obtained via a percutaneous transhepatic approach. The typical cholangiographic findings of PSC include multifocal beading of both the intrahepatic and the extrahepatic biliary tree. The intrahepatic bile ducts alone or of the extrahepatic bile ducts alone can be involved. Strictures are typically diffusely

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  • Cited by (0)

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