Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC).
OBJECTIVE
This study aims at describing the natural history and outcome of small duct primary sclerosing cholangitis (PSC).


METHODS
Thirty-two patients with small duct PSC were studied. The average time taken for diagnosis was 69 (1-168) months. The median follow-up time was 63 (1-194) months.


RESULTS
All patients including one who underwent liver transplantation because of end-stage liver disease and hepatocellular carcinoma were alive at follow-up. None developed cholangiocarcinoma. In 27 patients repeated cholangiographic examinations were done after a median time of 72 (12-192) months from first ERCP. Four developed features of large duct PSC.


CONCLUSIONS
Small duct PSC rarely progresses to large bile duct PSC and it seems to have a benign course in most patients and no development of cholangiocarcinoma was found.
Broomé
Ulrika
U
Department of Gastroenterology, Huddinge University Hospital, Stockholm, Sweden. ulrika.broome@medhs.ki.se
Glaumann
Hans
H
Lindstöm
Eva
E
Lööf
Lars
L
Almer
Sven
S
Prytz
Hanne
H
Sandberg-Gertzén
Hanna
H
Lindgren
Stefan
S
Fork
Frans-Thomas
FT
Järnerot
Gunnar
G
Olsson
Rolf
R
D000293:Adolescent; D000328:Adult; D000368:Aged; D000963:Antimetabolites / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D001650:Bile Duct Neoplasms / Q000453:epidemiology; D017734:Bile Ducts, Extrahepatic; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000453:epidemiology; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors; D013256:Steroids / Q000627:therapeutic use; D013548:Sweden / Q000453:epidemiology; D016896:Treatment Outcome
D000963:Antimetabolites; D013256:Steroids; D001379:Azathioprine