Progression to Cirrhosis, Cholangiocarcinoma, and Need for Liver Transplantation in Patients With Primary Sclerosing Cholangitis
Document Type
Conference Proceeding
Publication Date
5-2024
Publication Title
Gastroenterology
Abstract
Introduction: Primary sclerosing cholangitis (PSC) is a chronic progressive disease of the bile ducts leading to inflammation and fibrosis of the liver. PSC is a rare and highly heterogenous disease with variable rates of progression to cirrhosis and the need for liver transplantation (LT). Due to rarity of PSC, data on the natural history and outcomes of patients with PSC remain significant to report. We aimed to analyze the characteristics of patients with PSC at our center. Methods: We performed a retrospective review of patients with PSC at our institution between 1/2018-3/2023. Patient characteristics, the presence of cirrhosis, cholangiocarcinoma (CCA), and need for LT were obtained from medical records. Variables are reported as median with first and third quartiles, or numbers with percentages. Results: A total of 135 patients (78 male, 57 female) were included in the study. The majority of patients were Caucasian (n=91, 67.4%), following African American (n=40, 29.6%), and Asian (n=4, 2.9%). Fifty-one (37.7%) patients had cirrhosis (table 1), of which 21 (15.5%) underwent LT in a median of 8.7 (3.8-15.8) years from the diagnosis of PSC. Eighty-eight (65.1%) patients had concomitant inflammatory bowel disease (IBD). Eleven (8.1%) patients developed CCA over a median period of 12.6 (0.15-27.2) years. Median age at PSC diagnosis was 40.3 (28.8-57.1) years, at cirrhosis 56.1 (35.2-70.7) years, and at CCA diagnosis 42.3 (30.8-75.9) years. Median time from diagnosis of PSC to development of cirrhosis was 4.9 (0.75-13.3) years, and to CCA 12.6 (0.15-27.2) years. Thirty (22.2%) patients died during follow-ups, and median time from PSC diagnosis to death was 9.45 (3.95-15.7) years. Of patients who underwent LT, 4 (19.0%) had recurrent PSC in a median of 10.2 (5.48-15.7) years, and 1 patient required re-LT. Four (19.0%) patients died after LT in a median time of 3.10 (0.72-6.30) years. Patients who underwent LT were significantly older at death compared to those who had not [67.8 (42.9-79.1) vs 39.2 (29.0-53.6), p= 0.0483], and less commonly had ascending cholangitis (30.7% vs 66.6%, p=0.0007). No statistically significant differences were observed for gender, race, age at diagnosis of PSC, rates of cholangiocarcinoma, presence of IBD, or rate of death in patients who underwent LT compared to those who did not. Caucasian patients with PSC had significantly higher rate of concurrent IBD compared to African American patients (70.3% vs 52.5%, p=0.04), (table 2). Discussion: No age, gender or racial disparities for access to LT were observed at our center in patients with PSC. The presence of CCA was similar in patients with cirrhosis compared to those who did not. As PSC is rare and highly heterogenous, real-world data on progression and outcomes remain important to report.
Volume
166
Issue
5 Suppl
First Page
S1706
Last Page
S1707
Recommended Citation
Ozturk NB, Jamil LH. Progression to cirrhosis, cholangiocarcinoma, and need for liver transplantation in patients with primary sclerosing cholangitis. Gastroenterology. 2024 May;166(5 Suppl):S1706-S1707. doi: 10.1016/S0016-5085(24)04386-5
DOI
10.1016/S0016-5085(24)04386-5
Comments
DDW Digestive Disease Week, May 18-21, 2024, Washington, DC