In terms of recurrent PSC after LDLT, there is limited information of about posttransplant outcomes. Data from the A2ALL Study reported that PSC patients had a significantly higher survival rate compared to other disease etiologies [37]. Data from a Japanese survey study on 114 PSC patients all undergoing LDLT at 29 institutions reported a recurrence rate of 27 % and graft loss rates of 69 % in patients with recurrent disease. They reported the following as risk factors for recurrent disease in multivariate analysis: high MELD scores, first-degree-relative donors, postoperative CMV infection, and early biliary anastomotic complications [15]. However, since there is no DDLT comparator group, these data are difficult to place in context. An analysis of the SRTR registry compared patient and graft survival rates for autoimmune and cholestatic disease (including PSC) for LDLT vs. DDLT recipients. There was no difference in patient or graft survival rates for LDLT vs. DDLT in this cohort [26].

An uncommon indication for liver transplantation, PSC is associated with excellent long-term survival rates largely due to the relatively young age of recipients and their absence of comorbid medical conditions which could jeopardize the success of the operation. However, disease recurrence occurs in about one in six patients. The diagnosis may be suspected by elevated liver function tests, typically the alkaline phosphatase or bilirubin, and requires confirmation with either liver biopsy or cholangiography with ERCP or cross-sectional imaging. While numerous risk factors for recurrence have been reported, none have practical implications. There is no known therapy for recurrent disease that predictably changes its natural history. Most patients are administered ursodiol, and symptomatic cholangitis is treated with antibiotics and biliary drainage procedures, as indicated. Recurrent disease can be managed effectively, and graft loss requiring retransplantation does not occur in most cases.