TBA (16S149)

Morbidity and Mortality in Primary Sclerosing Cholangitis: 20 years of data from the National Liver Unit

Author(s)

O'Reilly S, Hartery K, Buckley M, Horgan G, Cullen G, Doherty G, MacNicholas R, McCormick A, Iqbal M, Houlihan D, Sheridan J

Department(s)/Institutions

Gastroenterology Dept, St Vincent's University Hospital; National Liver Unit, St Vincent's University Hospital

Introduction

There is a well established, yet still poorly understood link between PSC and inflammatory bowel disease (IBD). St Vincent’s University Hospital is the National Transplant Unit for the Republic of Ireland, as well as a large IBD specialist centre.

Aims/Background

To examine morbidity and mortality in a large PSC/transplant cohort, and to compare PSC alone to PSC/IBD.

Method

Data from HIPE, the National Liver Unit and the IBD database were used to create a database capturing all patients with PSC referred to St Vincent’s since the founding of the National Unit in 1994, and looking at basic patient demographics, distribution of disease, complications and mortality.

Results

102 (57.9%) have undergone liver transplant in the Republic of Ireland, with indications being cholangiocarcinoma, decompensated cirrhosis and recurrent cholangitis. Mean age at transplant was 46.3 years (Median 47 years, IQR 46.3-57.72) 19(10.7%) developed cholangiocarcinoma. Almost half of these (n=9) did not have concurrent IBD. Recurrence of PSC occurred in 19.6%. Median time to recurrence was 6.07 years (IQR 5.01-6.84). There was no difference in graft survival in those with recurrence vs non-recurrence. 40 of those with PSC/IBD required colectomy, 24 of whom also underwent transplant (10 colectomies pre-OLT, 14 post). Mortality rate in the whole cohort is 23.8% (n=42). 30.9% (n=13) from cholangiocarcinoma, hepatocellular carcinoma or post transplant lymphoproliferative disorder. 16.6% died due to post transplant complications (bleeding, pancreatitis, ischaemia, sepsis), and the remainder died from complications of cirrhosis. 73.8% of deaths occurred in patients who were post transplant. The mean follow up post transplant was 5.68 years (median 5.36, IQR 2.01-7.67). When post transplant deaths were divided into those with concurrent IBD (n=22)vs PSC alone (n=10), mean survival was 4.53 years (median 5.19, IQR 0.52-7.11) vs mean survival of 0.2 years (median 2.27, IQR 0.07-4.3), which was statistically significant(p <0.0001).

Conclusions

In this cohort, patients with concurrent IBD have improved survival. Little data has been published regarding this. Much work is needed regarding PSC and its long term management post transplant. Decreased survival rates may be attributable to immunosuppression regimes, recurrence, and the aggressive nature of cholangiocarcinoma.

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