Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis.
BACKGROUND
The present study was undertaken to identify the clinicopathological differences between sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) and primary sclerosing cholangitis (PSC).


METHODS
We retrospectively compared the clinical, cholangiographic, and liver biopsy findings between 24 cases of PSC and 24 cases of SC-AIP.


RESULTS
Patient age at the time of diagnosis was significantly lower in the PSC group than in the SC-AIP group. The peripheral blood eosinophil count was significantly higher in the PSC group than in the SC-AIP group, but the serum IgG4 level was significantly higher in the SC-AIP group. Cholangiography revealed band-like strictures, beaded appearance, and pruned-tree appearance significantly more frequently in PSC, whereas segmental strictures and strictures of the distal third of the common bile duct were significantly more common in SC-AIP. Liver biopsy revealed fibrous obliterative cholangitis only in the PSC specimens. No advanced fibrous change corresponding to Ludwig's stages 3 and 4 was observed in any of the SC-AIP specimens. IgG4-positive plasma cell infiltration of the liver was significantly more severe in SC-AIP than in PSC. Subsequent cholangiography showed no improvement in any of the PSC cases, but all SC-AIP patients responded to steroid therapy, and improvement in the strictures was observed cholangio-graphically.


CONCLUSIONS
Based on the differences between the patients' ages and blood chemistry, cholangiographic, and liver biopsy findings, SC-AIP should be differentiated from PSC.
Nishino
Takayoshi
T
Institute of Gastroenterology, Department of Medicine, Tokyo Women's Medical University, School of Medicine, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan.
Oyama
Hiroyasu
H
Hashimoto
Etsuko
E
Toki
Fumitake
F
Oi
Itaru
I
Kobayashi
Makio
M
Shiratori
Keiko
K
D000328:Adult; D000367:Age Factors; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D001706:Biopsy; D002758:Cholangiography; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging / Q000379:methods; D004804:Eosinophils; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000378:metabolism; D007150:Immunohistochemistry; D007958:Leukocyte Count; D008099:Liver / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010179:Pancreas / Q000473:pathology; D010195:Pancreatitis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D012189:Retrospective Studies
D007074:Immunoglobulin G