This article focusses on the potential reoccurrence of symptoms after liver transplant in PFIC 2 patients. PFIC 2 is caused by mutations in the bile salt export pump (BSEP), leading BSEP to function poorly or not at all (BSEP deficiency). This in turns leads to build up of bile salts in the liver (cholestasis). Depending on severity, liver transplant is often the only working treatment option. However, some PFIC 2 patients seem to develop BSEP deficiency again after liver transplant.
The researchers investigated the potential causes in 9 patients who had undergone liver transplantation previously, and showed that some of these patients had developed antibodies that inhibit BSEP between 3 and 108 months post-transplant. This is called antibody induced BSEP deficiency, AIBD, and it seems to occur in severely affected patients who have complete BSEP deficiency before transplant.
PFIC 2 Post-transplant, BSEP deficiency, AIBD
Stindt et al. Bile Salt Export Pump-Reactive Antibodies Form a Polyclonal, Multi-Inhibitory Response in Antibody Induced Bile Salt Export Pump Deficiency. Journal: Hepatology, Vol. 63, No. 2 Year: 2016.