Livmarli is a medicine used for treating patients aged 2 months and older with cholestatic pruritus (intense itching due to a build-up of bile) caused by Alagille syndrome. It is also used for treating patients aged 3 months and older with progressive familial intrahepatic cholestasis. Alagille syndrome and progressive familial intrahepatic cholestasis are inherited diseases in which bile (a fluid produced in the liver that helps to break down fats) cannot drain properly from the liver, resulting in a build-up of bile acid in the liver and blood. One of the symptoms of this build-up is cholestatic pruritus. Intrahepatic cholestasis is associated with progressive liver damage and can lead to cirrhosis and end-stage liver impairment. These diseases are rare, and Livmarli was designated an ‘orphan medicine’ (a medicine used in rare diseases). Further information on the orphan designations can be found on the EMA website ( [Alagille syndrome](https://www.ema.europa.eu/en/medicines/human/orphan-designations/eu-3-13-1214); [progressive familial intrahepatic cholestasis](https://www.ema.europa.eu/en/medicines/human/orphan-designations/eu-3-13-1216)). Livmarli contains the active substance maralixibat chloride.
Therapeutic Indication
### Therapeutic indication Livmarli is indicated for the treatment of: - Cholestatic pruritus in patients with Alagille syndrome (ALGS) 2 months of age and older, - Progressive familial intrahepatic cholestasis (PFIC) in patients 3 months of age and older. Livmarli tablets is indicated in adults and adolescents 12 years and older for the treatment of: - Cholestatic pruritus in patients with Alagille syndrome (ALGS), - Progressive familial intrahepatic cholestasis (PFIC).
Therapeutic Area (MeSH)
ATC Code
A05AX04
ATC Item
maralixibat chloride
Pharmacotherapeutic Group
Other drugs for bile therapy
Active Substance (Summary)
INN / Common Names
| Substance | CAS | Monograph |
|---|---|---|
| Maralixibat chloride | N/A | 氯马昔巴特 |
EMA Name
Livmarli
Medicine Name
Livmarli
Aliases
N/A