Composition for the treatment of cystic fibrosis

Abstract

The present invention provides compositions comprising a prostacyclin or prostacyclin analogue, or a pharmaceutically acceptable salt thereof for use in preventing or treating cystic fibrosis. The invention also provides the use of a kit comprising a prostacyclin or prostacyclin analogue for treating or preventing a condition associated with cystic fibrosis in a subject.

1 . A composition comprising a prostacyclin or an analogue, derivative, or pharmaceutically acceptable salt thereof for use in preventing or treating cystic fibrosis. 
     
     
         2 . The composition according to  claim 1 , wherein said prostacyclin analogue is selected from the group consisting of treprostinil, iloprost, cisaprost or beraprost or a derivative or pharmaceutically acceptable salt thereof. 
     
     
         3 . The composition according to  claim 1 , wherein said derivative is selected from the group consisting of an acid derivative of treprostinil, a prodrug of treprostinil, a sustained release form of treprostinil, an inhaled form of treprostinil, an oral form of treprostinil, a polymorph of treprostinil, and an isomer treprostinil. 
     
     
         4 . The composition according to  claim 1 , wherein the composition is in a form suitable for intravenous administration. 
     
     
         5 . The composition according to  claim 1 , wherein the composition is in a form suitable for inhalation. 
     
     
         6 . The composition according to  claim 1 , wherein said composition is in an orally available form selected from the group consisting of tablets and capsules. 
     
     
         7 . The composition according to  claim 1 , wherein the effective amount of treprostinil or its derivative or pharmaceutically acceptable salt thereof is at least 1.0 ng/kg of body weight/min. 
     
     
         8 . A method for treating or preventing a condition associated with cystic fibrosis in a subject, comprising administering to the subject an effective amount of a compound comprising prostacyclin or a prostacyclin analogue or a derivative or pharmaceutically acceptable salt thereof together with one or more pharmaceutically acceptable carriers and/or additives. 
     
     
         9 . The method of  claim 8 , wherein the compound is a pharmaceutically acceptable salt of treprostinil. 
     
     
         10 . The method of  claim 8 , wherein the compound is in a form suitable for intravenous administration. 
     
     
         11 . The method of  claim 8 , wherein the compound is in a form suitable for inhalation. 
     
     
         12 . The method of  claim 8 , wherein the compound is in a form suitable for oral administration. 
     
     
         13 . The method of  claim 12 , wherein the compound is in a form selected from the group consisting of tablets and capsules. 
     
     
         14 . The method of  claim 8 , wherein the compound is a prostacyclin analogue selected from the group consisting of treprostinil, iloprost, cisaprost, beraprost and a derivative or pharmaceutically acceptable salt thereof. 
     
     
         15 . The method of  claim 8 , wherein the composition is administered in an amount of at least 1.0 ng/kg of the subject's body weight/minute. 
     
     
         16 . The method of  claim 8 , wherein the compound is a prostacyclin derivative selected from the group consisting of an acid derivative of treprostinil, a prodrug of treprostinil, a sustained release form of treprostinil, an inhaled form of treprostinil, an oral form of treprostinil, a polymorph of treprostinil and an isomer of treprostinil.