Clinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis.
OBJECTIVE
Sclerosing cholangitis (SC) with autoimmune pancreatitis (AIP) is similar to primary sclerosing cholangitis (PSC) with regard to cholangiographic findings, but only the former responds well to steroid therapy. This report concentrates on the clinical differences between these diseases.


METHODS
The presenting complaint or abnormality, associated disease, cholangiographic findings, pancreatic changes, treatment, and clinical course were studied for several cases of PSC (n = 27) and SC with AIP (n = 20).


RESULTS
SC with AIP as a diagnosis occurs abruptly with obstructive jaundice compared with PSC where diagnosis is often based on findings of asymptomatic liver test abnormalities. In addition, inflammatory bowel disease is only associated with PSC. The most prominent features of cholangiograms for the SC with AIP cases were stenosis of the lower common bile duct. However, sclerosing changes in the intra- and extrahepatic bile ducts or hilar hepatic region were observed for half of the cases. Only PSC showed stage III or IV liver biopsy findings. IgG4 was significantly higher in SC with AIP.


CONCLUSIONS
SC with AIP and PSC are different clinical entities.
Nakazawa
Takahiro
T
Department of Internal Medicine and Bioregulation, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan. tnakazaw@med.nagoya-cu.ac.jp
Ohara
Hirotaka
H
Sano
Hitoshi
H
Ando
Tomoaki
T
Aoki
Shigeru
S
Kobayashi
Shinya
S
Okamoto
Tetsu
T
Nomura
Tomoyuki
T
Joh
Takashi
T
Itoh
Makoto
M
D000328:Adult; D017668:Age of Onset; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications / Q000473:pathology* / Q000628:therapy; D001663:Bilirubin / Q000097:blood; D001706:Biopsy; D002758:Cholangiography; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000473:pathology* / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D015212:Inflammatory Bowel Diseases / Q000150:complications / Q000473:pathology; D008099:Liver / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010179:Pancreas / Q000473:pathology; D050500:Pancreatitis, Chronic / Q000150:complications / Q000473:pathology* / Q000628:therapy; D011379:Prognosis
D007074:Immunoglobulin G; D001663:Bilirubin