Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis.
OBJECTIVE
To clarify the characteristic features of biliary lesions in patients with autoimmune pancreatitis (AIP) and compare them with those of primary sclerosing cholangitis (PSC).


METHODS
The clinicopathological characteristics of 34 patients with sclerosing cholangitis (SC) associated with AIP were compared with those of 4 patients with PSC.


RESULTS
SC with AIP occurred predominantly in elderly men. Obstructive jaundice was the most frequent initial symptom in SC with AIP. Only SC patients with AIP had elevated serum IgG4 levels, and sclerosing diseases were more frequent in these patients. SC patients with AIP responded well to steroid therapy. Segmental stenosis of the lower bile duct was observed only in SC patients with AIP, but a beaded and pruned-tree appearance was detected only in PSC patients. Dense infiltration of IgG4-positive plasma cells was detected in the bile duct wall and the periportal area, as well as in the pancreas, of SC patients with AIP.


CONCLUSIONS
SC with AIP is distinctly different from PSC. The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.
Kamisawa
Terumi
T
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, Japan. kamisawa@cick.jp
Takuma
Kensuke
K
Anjiki
Hajime
H
Egawa
Naoto
N
Kurata
Masanao
M
Honda
Goro
G
Tsuruta
Kouji
K
D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications*; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007150:Immunohistochemistry; D008297:Male; D010195:Pancreatitis / Q000150:complications*
D007074:Immunoglobulin G