Ibat inhibitors for the treatment of liver diseases

Abstract

The present invention regards specific IBAT inhibitors useful in the prophylaxis and/or treatment of a liver disease. It also relates to compositions comprising these IBAT inhibitors, a method for treatment of the disorders and a kit comprising the substances or the compositions.

1 . (canceled) 
     
     
         2 . A method for treating primary biliary cirrhosis (PBC) comprising orally administering to a subject in need of such treatment a therapeutically effective amount of an IBAT inhibitor, or a pharmaceutically acceptable salt thereof, and ursodeoxycholic acid. 
     
     
         3 . The method of  claim 2 , wherein the IBAT inhibitor and the ursodeoxycholic acid are administered simultaneously. 
     
     
         4 . The method of  claim 2 , wherein the IBAT inhibitor and the ursodeoxycholic acid are administered separately. 
     
     
         5 . The method of  claim 2 , wherein the IBAT inhibitor and the ursodeoxycholic acid are administered sequentially. 
     
     
         6 . The method of  claim 2 , wherein treatment of primary biliary cirrhosis comprises treatment of pruritis. 
     
     
         7 . The method of  claim 2 , wherein treatment of primary biliary cirrhosis comprises decreasing the level of serum bile acids in the subject. 
     
     
         8 . The method of  claim 2 , wherein the IBAT inhibitor is not systemically absorbed. 
     
     
         9 . A method for treating a liver disease selected from the group consisting of primary biliary cirrhosis (PBC); Alagille syndrome (ALGS); primary sclerosing cholangitis (PSC); and progressive familial intrahepatic cholestasis (PFIC), the method comprising orally administering to a subject in need of such treatment a therapeutically effective amount of an IBAT inhibitor, or a pharmaceutically acceptable salt thereof, and ursodeoxycholic acid. 
     
     
         10 . The method of  claim 9 , wherein the IBAT inhibitor and the ursodeoxycholic acid are administered simultaneously. 
     
     
         11 . The method of  claim 9 , wherein the IBAT inhibitor and the ursodeoxycholic acid are administered separately. 
     
     
         12 . The method of  claim 9 , wherein the IBAT inhibitor and the ursodeoxycholic acid are administered sequentially. 
     
     
         13 . The method according to  claim 9 , wherein the liver disease is primary biliary cirrhosis (PBC). 
     
     
         14 . The method according to  claim 9 , wherein the liver disease is Alagille syndrome (ALGS) 
     
     
         15 . The method according to  claim 9 , wherein the liver disease is progressive familial intrahepatic cholestasis (PFIC). 
     
     
         16 . The method of  claim 15 , wherein the progressive familial intrahepatic cholestasis is Byler syndrome. 
     
     
         17 . The method of  claim 15 , wherein the progressive familial intrahepatic cholestasis is PFIC 1. 
     
     
         18 . The method according to  claim 9 , wherein the liver disease is primary sclerosing cholangitis (PSC). 
     
     
         19 . The method of  claim 9 , wherein treatment of the liver disease comprises treatment of pruritis. 
     
     
         20 . The method of  claim 9 , wherein treatment of the liver disease comprises decreasing the level of serum bile acids in the subject. 
     
     
         21 . The method of  claim 9 , wherein the IBAT inhibitor is not systemically absorbed. 
     
     
         22 - 24 . (canceled)