P35 Outcomes in children with primary sclerosing cholangitis or autoimmune hepatitis-overlap and associated inflammatory bowel disease

Background

Primary Sclerosing Cholangitis (PSC) and Autoimmune liver disease (autoimmune hepatitis & overlap syndrome (AIHO)) are rare entities. PSC and AIHO have been reported to be associated with IBD.

Aim

To study outcomes of children diagnosed with AIHO and PSC who also have a diagnosis of inflammatory bowel disease (IBD). Outcomes included portal hypertension, biochemical remission, survival of native liver and mortality.

Methods

This is a retrospective study (2000–2020) of 193 patients diagnosed with AIH. Of these, 23 patients (14M:9F) had diagnosis of either AIHO+IBD or PSC+IBD. None had AIH without overlap. Case notes were examined at intervals of 1,3,5,7 and 10 years. Data included treatment modality, biochemical remission (ALT <50 iu/l), GGT (<40 iu/l), development of portal hypertension (platelet count <120, splenomegaly and varices) and survival of native liver.

Results

23 (12%) patients were identified, of which 14 (60%) had a diagnosis of PSC+ IBD (Crohn’s: 6, indeterminate: 7, UC:1). Of the PSC group, 35% had gastric, duodenal and colonic disease with 1 patient undergoing pan-proctocolectomy. All but one were diagnosed with IBD prior to PSC or concomitantly. The remaining 40% patients had AIHO+IBD (UC:7, indeterminate: 2). AIHO was diagnosed prior to IBD in 4; 3 were diagnosed with IBD prior to AIH. In this group, 66% had severe pancolitis and 33% required surgical resection. (Results summarised in table below). In the PSC+IBD group follow-up data included 1 year (n =8), 3 year (n=6), 5 year (n =5) 7 year (n =3). Median age at diagnoses was 9 years (5–16). Aside from one patient, patients were diagnosed with IBD prior to PSC or concomitantly. At diagnosis, 64% received ursodeoxycholic acid; 21% had steroids, 14% had azathioprine and 57% had aminosalicylates. Two patients received infliximab and adalimumab. The median Gamma GT (GGT) at diagnosis was 268 (16–877), at 1 year was 37 (7–104), at 7 years was 31 (11–52). In the AIHO+IBD group, data included 1 year (n=8), 3 year (n=3), 5 year (n=1) and 7 year (n=1). Median age at diagnoses was 12 years (6–16). AIH was diagnosed prior to IBD in 4 patients; 3 patients were diagnosed with IBD prior to AIH. At diagnosis, 55% received ursodeoxycholic acid, 100% received steroids, 44% received azathioprine and 22% of patients received aminosalicylates. One year after diagnosis, one patient received infliximab. Biochemical remission was achieved in 75% (5) by 1 year and 100% (3) by three years. In both groups, normalisation of GGT or ALT did not correlate with resolution of endothelial thickening of the CBD on ultrasound. Portal hypertension was found in 13% of patients (PSC group 2; AIHO 1). No patients died or received a liver transplant.

Summary and Conclusion

Patients with PSC were more likely to have a diagnosis of IBD-U whilst patients with AIH-overlap were more often diagnosed with UC. In terms of IBD outcomes 13% of patients required biologics and surgical resection. In keeping with the literature, only one patient had portal hypertension at diagnosis. In total, 13% of AIHO+IBD and PSC+IBD patients developed portal hypertension. None died or required liver transplantation.