Background/Aims: We encountered one group of primary sclerosing cholangitis
cases which met classic criteria, but demonstrated better clinical courses
.
Methodology: We evaluated the clinical characteristics of several cases of
primary sclerosing cholangitis, especially those associated with chronic pa
ncreatitis.
Results: Of a total of 25 cases, eight had a background of pancreatitis wit
h unusual irregularly segmental or diffuse narrowing features on pancreatog
raphy. Abdominal ultrasonography and computed tomography also revealed segm
ental or diffuse enlargement of the pancreas. Pathological findings of surg
ical specimens in two cases pointed to lymphoplasmacytic sclerosing pancrea
titis. The walls of the lower biliary ducts were also thickened with a simi
lar appearance to the pancreatic ducts. In seven cases, cholangiography dis
played stenosis of the lower part of the common bile duct and in one case,
only the hepatic hilar region was affected. In this case both biliary and p
ancreatic lesions responded to steroid therapy simultaneously. One case dev
eloped hepatic hilar stenosis four months after a lesion in the lower part
of the common bile duct had disappeared. The cases of primary sclerosing ch
olangitis with pancreatitis demonstrated better clinical courses than did t
ypical cases of primary sclerosing cholangitis. Six cases were characterize
d by autoimmunological abnormalities.
Conclusions: We concluded that similar etiological agents might impact on b
oth the pancreas and biliary tract, either simultaneously or in sequence. W
e propose new criteria for these atypical primary sclerosing cholangitis ca
ses.