Abstracts of the 2022 Annual Meeting of the ALEH
More infoPrimary sclerosing cholangitis (PSC) is a cholestatic disease that commonly affects young males with inflammatory bowel disease (IBD). There is no efficient medical treatment, being orthotopic liver transplantation (OLT) the only curative treatment recommended in decompensated cirrhosis, intractable pruritus and recurrent cholangitis.
ObjectivesDescribe clinical, laboratory and histological findings in patients with PSC-IBD of a quaternary hospital and identify prognostic factors for OLT.
Materials and MethodsReview of patients’ medical records with PSC-IBD followed from 01/2000 to 05/2022, excluding cases with insufficient data.
ResultsAmong 73 patients, 57% were male; the mean age during PSC diagnosis was 34,2±14,3 years, with a follow-up period of 8,8±5.4 years, 85% of those presenting ulcerative colitis. During diagnosis, 93% were symptomatic, usually presenting with pruritus and fatigue. A liver biopsy was performed in 30 patients, and 60% of those revealed F3/4. 4 patients presented dominant strictures (DS) and 68 were treated with ursodeoxycholic acid (UDCA) 16mg/kg/d. 16 (21.9%) underwent OLT in a period of 6.3±4,5 years after diagnosis; the main indication was decompensated cirrhosis. Ten patients had cancer, and the two most frequent were colorectal carcinoma and cholangiocarcinoma. 13 patients died; from those, four were transplanted and six died of infection. Between patients with and without OLT, there were no significant differences in age during diagnosis, type of IBD, comorbidities, presence of symptoms during diagnosis, histological fibrosis, or presence of DS. The OLT group had higher levels of bilirubin (3 × 0,8mg/dL;p<0,001) and lower albumin levels (3,4 × 4,3g/dL;p<0,001) during diagnosis. They also presented higher levels of alkaline phosphatasis (394 × 223U/L;p<0,001) and lower frequency of normalization (0 × 30%;p=0,027) after 6-month treatment with UDCA.
ConclusionsHigher levels of bilirubin and lower albumin during diagnosis and higher levels of alkaline phosphatasis after 6-month treatment with UDCA were associated with an increased risk of disease progression for OLT.