Læknablaðið - sep. 2019, Side 16

376 LÆKNAblaðið 2019/105
R A N N S Ó K N
Background: Primary sclerosing cholangitis (PSC) is a chronic cho-
lestatic disease affecting the intra- and/or extrahepatic biliary tree
with inflammation and progressive stricture formation that can lead to
cirrhosis, end stage liver failure and liver transplantation. Known risk
factors include inflammatory bowel diseases (IBD), mainly ulcerative
colitis (UC). Highest reported incidence in an adult population is 1.2-
1.3/100.000 in Norway and Sweden, where 60-76% have IBD. The aim
of this study was to investigate epidemiology of PSC in Iceland in the
years 1992 to 2012 and the patients outcomes.
Methods: A search for the diagnosis “cholangitis“ (ICD-10, K83.0)
was performed in the database for hospital records in Landspítali (The
National University Hospital of Iceland, LSH) and Akureyri Hospital from
1992 to 2012. We also looked through all ERCP and MRCP
imaging done in LSH in the same period along with a text search in both
the hospital records and the pathology database for liver biopsies. Data
on these patients was collected until the end of 2016.
Results: A total of 42 patient got the diagnosis PSC within the period.
Median age at diagnosis was 34 years, 67% were male and 90% adults
(≥18 years old). Mean incidence per year was 0.69/100.000. Overall
88% of patients had IBD, thereof 89% UC. Seven patients have been
diagnosed with cancer, four with cancer in the bile ducts and one in the
gallbladder. Within the study period a total of five patients died (12%),
51 months (median) from diagnosis and three from cholangiocarcinoma,
51 months (median) from diagnosis. Three patients (7%) underwent liver
transplantation, one required a transplant two times.
Conclusions: The incidence of PSC in Iceland turned out to be lower
than in our neighbouring countries in Scandinavia. It is unclear if this is
due to underdiagnosis or, more likely, that PSC is simply more uncom-
mon in Iceland. Overall 7% underwent liver transplantation and 12%
died within the study period, main cause of mortality being cholangio-
carcinoma.
Primary sclerosing cholangitis in Iceland 1992-2012
ENGLISH SUMMARY
1Department of Internal Medicine, Division of Gastroenterology, Landspítali University Hospital, Reykjavík, Iceland, 2Department of Pathology, Landspítali University Hospital,
Reykjavík, Iceland, 3Faculty of Medicine, University of Iceland, Reykjavík, Iceland.
Key words: PSC, primary sclerosing cholangitis, epidemiology, incidence, sclerosing cholangitis.
Correspondence: Hafsteinn Óli Guðnason, haddiog@gmail.com
Hafsteinn Óli Guðnason1
Jón Örvar Kristinsson1
Óttar Már Bergmann1
Sigurður Ólafsson1
Jón Gunnlaugur Jónasson2,3
Einar Stefán Björnsson1