Primary Schlerosing Cholangitis

My daughter has been treated for autoimmune hepatitis with cirrhosis for 3 years. Recently, her direct bilirubin tested at 6.2 with an ammonia level of 207. Liver enzymes were slightly elevated.  Her physician performed an ERCP and placed a stent. After reviewing the radiology reports, he said her diagnosis had been changed from AIH to primary sclerosing cholangitis.  She will be evaluated by a Dallas Liver Transplant Team at the end of October. What should we expect in terms of changes in her health between now and when they ultimately determine if and when she is going to be listed for a transplant?

[Thomas D Schiano, MD]

she appears to have an overlap syndrome between AIH and PSC, which is common amongst children.  if the bilirubin elevation is due to the latter and can not be treated with increased immunosuppression, then transplantation may be necessary in the near future.  she definitely should do well post-transplantation if it is necessary but both PSC and AIH can recur.  Starting ursodiol may help the PSC component of the overlap.