AIH, younger age increase risk for PSC recurrence in pediatric LT patients

CHICAGO — Researchers found that nearly a quarter of pediatric patients who underwent liver transplantation for primary sclerosing cholangitis had recurrence posttransplant, particularly patients who were younger or had autoimmune hepatitis, according to a presentation at Digestive Disease Week.

“Liver transplantation is the only therapy for primary sclerosing cholangitis. Most liver transplant outcomes for children are generally good. Pediatric onset PSC and PSC with autoimmune hepatitis overlap phenotype are quite rare. Furthermore, pediatric liver transplant for PSC is quite uncommon,” Fateh Bazerbachi, MD, from the Mayo Clinic, Rochester, Minnesota said. “Our study objective was to characterize the current PSC as [indication for] liver transplant using a large, multicenter international cohort of children with this disease.”

The researchers identified 781 pediatric patients with PSC, 55% of whom were boys. Mean age at initial PSC diagnosis was 10.5 years. Of the patients who underwent liver transplant for PSC, 92 were available for follow-up. Mean age at transplant was 14.8 years and follow-up after transplant was approximately 4 years.

Eighty-five of the liver transplant patients had inflammatory bowel disease (83% ulcerative colitis and 17% Crohn’s disease), while 30% of the patients had autoimmune hepatitis and 27% had small duct phenotype.

Sixteen of the 92 patients had PSC recurrence. Bazerbachi noted that while mean age at initial diagnosis was similar between those who had recurrence posttransplant and those who did not, the mean time of 2.2 years (range, 0.5-3.9 years) from initial diagnosis to transplantation was shorter among the patients with recurrence.

Five-year probability of PSC recurrence posttransplant was 25% (95% CI, 15-37). Mean age at transplantation was also lower in those with recurrence (12 vs. 15 years; P = .034) and patients with recurrence had a higher prevalence of autoimmune hepatitis (63% vs. 26%; P = .005). There were no differences in sex, IBD prevalence or small vs. large duct phenotype between the patients with recurrence and those without.
by Talitha Bennett

References:

Bazerbachi F, et al. Abstract 572. Presented at: Digestive Disease Week; May 6-9, 2017; Chicago.

Disclosure: Bazerbachi reports no relevant financial disclosures.