Use of maralixibat for severe pruritus in a post-liver transplant patient with ischaemic cholangiopathy

Pruritus is a common and sometimes severe symptom of cholestatic liver disease and can occur following liver transplantation. Pharmacological options for cholestasis-associated pruritus can be ineffective or poorly tolerated, with their own specific challenges in the transplant setting. Ileal bile acid transporter (IBAT) inhibitors, including maralixibat, are promising therapies for genetic cholestatic conditions. We describe the first use of maralixibat in a 63-year-old man post-liver transplant for intractable pruritus secondary to ischaemic cholangiopathy. Bezafibrate, naltrexone, ursodeoxycholic acid, cholestyramine and antihistamines were trialled over 11 months with no clinical benefit. Compassionate use of maralixibat was initiated, resulting in significant improvement in pruritus and quality of life. Liver graft function remained stable, and no interactions were observed with tacrolimus-based immunosuppression. Serum bile acid levels fell from 135 µmol/L to 26 µmol/L. Maralixibat was safe and effective in this setting. Further work is needed to ascertain the safety and efficacy of IBAT inhibitors post-liver transplant.