INNGANGUR Frumkomin trefjunargallgangabólga er langvinnur bólgusjúkdómur í gallvegum innan og/eða utan lifrar sem getur valdið skorpulifur, lokastigs lifrarbilun og leitt til lifrarígræðslu. Bólgusjúkdómar í meltingarvegi, fyrst og fremst sáraristilbólga, er algengur áhættuþáttur. Hæsta nýgengi fullorðinna sem hefur verið birt var 1,2- 1,3/100.000 í Noregi og Svíþjóð og 60-76% höfðu bólgusjúkdóm í meltingarvegi. Markmið þessarar fyrstu rannsóknar sjúkdómsins á Íslandi var að kanna faraldsfræði hans frá árunum 1992-2012 og afdrif sjúklinganna. AÐFERÐIR Leit var framkvæmd í gagnagrunnum Landspítala og Sjúkrahússins á Akureyri að sjúkdómsgreiningunni: K83.0, „Gallgangabólga“, frá 1992 til 2012. Að auki var gerð leit að sjúklingum með yfirferð á öllum gallvegaspeglunum og segulómunum af gallvegum sem framkvæmdar voru á Landspítala 1992-2012. Einnig var gerð textaleit bæði í gagnagrunnum beggja spítalanna og í gagnagrunni meinafræðinnar fyrir lifrarsýni. NIÐURSTÖÐUR Alls fundust 42 sjúklingar með sjúkdóminn innan umrædds tímabils. Miðgildi aldurs við greiningu var 34 ára, 67% voru karlkyns og 90% fullorðnir (≥18 ára). Meðalnýgengi á ári var 0,69/100.000 manns á rannsóknartímabilinu. Alls 88% sjúklinga reyndust vera með bólgusjúkdóm í meltingarvegi, þar af 89% sjúklinga með sáraristilbólgu. Sjö sjúklingar hafa verið greindir með krabbamein, þar af fjórir með meinið í gallgöngum og einn í gallblöðru. Innan tímabilsins dóu 5 sjúklingar (12%), 51 mánuði (miðgildi) frá greiningu og þar af þrír úr gallgangakrabbameini 51 mánuðum (miðgildi) frá greiningu. Þrír (7%) þurftu lifrarígræðslu, þar af einn í tvígang. ÁLYKTANIR Nýgengi á Íslandi reyndist lægra en í nágrannalöndum okkar í Skandinavíu. Það er óljóst hvort það stafar af vangreiningu tilfella og/eða að sjúkdómurinn sé sjaldgæfari á Íslandi en í Noregi og Svíþjóð. Alls 7% þurftu á lifrarígræðslu að halda og 12% dóu úr sjúkdómnum, aðallega vegna gallgangakrabbameins. Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic 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 uncommon in Iceland. Overall 7% underwent liver transplantation and 12% died within the study period, main cause of mortality being cholangiocarcinoma.
Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic 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 uncommon in Iceland. Overall 7% underwent liver transplantation and 12% died within the study period, main cause of mortality being cholangiocarcinoma.
