US2014163068A1PendingUtilityA1

Pharmaceutical Compositions for the Treatment of CFTR Mediated Diseases

Assignee: VERTEX PHARMAPriority: Nov 2, 2012Filed: Nov 1, 2013Published: Jun 12, 2014
Est. expiryNov 2, 2032(~6.3 yrs left)· nominal 20-yr term from priority
A61P 43/00A61P 3/12A61P 1/16A61P 1/00A61P 11/00A61P 1/18A61K 31/443A61K 9/20A61K 9/2027A61K 31/47A61K 9/14A61K 9/2013A61K 9/2095A61K 9/2054A61K 9/2077A61K 9/0053A61K 9/10A61K 45/06A61K 9/1605A61K 9/2004
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Claims

Abstract

Pharmaceutical compositions comprising 3-(6-(1-(2,2-difluorobenzo[d][1,3]dioxol-5-yl)cyclopropanecarboxamido)-3-methylpyridin-2-yl)benzoic acid (Compound 1) in Form I and a solid dispersion comprising substantially amorphous N-(5-hydroxy-2,4-ditert-butyl-phenyl)-4-oxo-1H-quinoline-3-carboxamide (Compound 2), methods of treating, lessening the severity of, or symptomatically treating CFTR mediated diseases, such as cystic fibrosis, methods of manufacturing, methods of administering, and kits thereof are disclosed.

Claims

exact text as granted — not AI-modified
1 . A pharmaceutical composition comprising a fixed dosage amount of 3-(6-(1-(2,2-difluorobenzo[d][1,3] dioxol-5-yl)cyclopropanecarboxamido)-3-methylpyridin-2-yl)benzoic acid (Compound 1) Form I and a solid dispersion comprising substantially amorphous N-(5-hydroxy-2,4-ditert-butyl-phenyl)-4-oxo-1H-quinoline-3-carboxamide (Compound 2). 
     
     
         2 . The pharmaceutical composition of  claim 1  further comprising:
 a. a filler; 
 b. a disintegrant; 
 c. a surfactant; and 
 d. a binder; 
 
       referred to as PC-I. 
     
     
         3 . The pharmaceutical composition of  claim 1 , comprising 30 to 55 percent by weight Compound 1 Form I, and 10 to 45 percent by weight solid dispersion comprising substantially amorphous Compound 2. 
     
     
         4 . The pharmaceutical composition of  claim 2  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   35-50 
                 
                     
                   Solid dispersion comprising 
                   25-40 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   10-20 
                 
                     
                   Croscarmellose sodium 
                   1-3 
                 
                     
                   Sodium lauryl sulfate 
                   0.5-2   
                 
                     
                   Polyvinylpyrrolidone 
                   0-5 
                 
                     
                     
                 
             
                
                
                
               
               
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-II. 
     
     
         5 . A pharmaceutical composition comprising:
 a. Compound 1 Form I;   b. a solid dispersion comprising substantially amorphous Compound 2;   c. a filler;   d. a disintegrant;   e. a surfactant;   f. a binder; and   g. a lubricant;   
       referred to as PC-III. 
     
     
         6 . The pharmaceutical composition of  claim 5 , comprising about 100 to 250 mg of Compound 1 Form I, and about 80 to 150 mg of substantially amorphous Compound 2. 
     
     
         7 . The pharmaceutical composition of  claim 5 , comprising about 200 mg of Compound 1 Form I, and about 125 mg of substantially amorphous Compound 2. 
     
     
         8 . The pharmaceutical composition of  claim 5 , comprising about 200 mg of Compound 1 Form I, and about 83 mg of substantially amorphous Compound 2. 
     
     
         9 . The pharmaceutical composition of  claim 5 , comprising about 150 mg of Compound 1 Form I, and about 125 mg of substantially amorphous Compound 2. 
     
     
         10 . The pharmaceutical composition of  claim 5 , comprising 25 to 50 percent by weight Compound 1 Form I, and 15 to 35 percent by weight a solid dispersion comprising substantially amorphous Compound 2. 
     
     
         11 . The pharmaceutical composition of  claim 5  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   25-50 
                 
                     
                   A solid dispersion comprising 
                   15-35 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   20-30 
                 
                     
                   Croscarmellose sodium 
                    3-10 
                 
                     
                   Sodium lauryl sulfate 
                   0.5-2   
                 
                     
                   Polyvinylpyrrolidone 
                   0-5 
                 
                     
                   Magnesium stearate 
                   0.5-2   
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-IV. 
     
     
         12 . The pharmaceutical composition of  claim 5 , further comprising a colorant and a wax. 
     
     
         13 . The pharmaceutical composition of  claim 2 , wherein the pharmaceutical composition is a solid oral pharmaceutical composition. 
     
     
         14 . The pharmaceutical composition  claim 13 , wherein the solid oral pharmaceutical composition is a granule. 
     
     
         15 . The granule of  claim 14  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   43 
                 
                     
                   Solid dispersion comprising 
                   34 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   17 
                 
                     
                   Croscarmellose sodium 
                   2 
                 
                     
                   Sodium lauryl sulfate 
                   1 
                 
                     
                   Polyvinylpyrrolidone 
                   3 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-V. 
     
     
         16 . The granule of  claim 14  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   38 
                 
                     
                   Solid dispersion comprising 
                   40 
                 
                     
                   substantially amorphous  
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   16 
                 
                     
                   Croscarmellose sodium 
                   2 
                 
                     
                   Sodium lauryl sulfate 
                   1 
                 
                     
                   Polyvinylpyrrolidone 
                   3 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-VI. 
     
     
         17 . The granule of  claim 14  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   51 
                 
                     
                   Solid dispersion comprising 
                   27 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   16 
                 
                     
                   Croscarmellose sodium 
                   2 
                 
                     
                   Sodium lauryl sulfate 
                   1 
                 
                     
                   Polyvinylpyrrolidone 
                   3 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
               
            
           
         
         referred to as PC-VII. 
       
     
     
         18 . The pharmaceutical composition of  claim 5 , wherein the pharmaceutical composition is a solid oral pharmaceutical composition. 
     
     
         19 . The pharmaceutical composition  claim 18 , wherein the solid oral pharmaceutical composition is a tablet. 
     
     
         20 . The tablet  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   35 
                 
                     
                   Solid dispersion comprising 
                   28 
                 
                     
                   substantially amorphous  
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   26 
                 
                     
                   Croscarmellose sodium 
                   6 
                 
                     
                   Sodium lauryl sulfate 
                   1 
                 
                     
                   Polyvinylpyrrolidone 
                   3 
                 
                     
                   Magnesium stearate 
                   1 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-VIII. 
     
     
         21 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   31 
                 
                     
                   Solid dispersion comprising 
                   32 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   26 
                 
                     
                   Croscarmellose sodium 
                   6 
                 
                     
                   Sodium lauryl sulfate 
                   1 
                 
                     
                   Polyvinylpyrrolidone 
                   3 
                 
                     
                   Magnesium stearate 
                   1 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-IX. 
     
     
         22 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   41 
                 
                     
                   Solid dispersion comprising 
                   22 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   26 
                 
                     
                   Croscarmellose sodium 
                   6 
                 
                     
                   Sodium lauryl sulfate 
                   1 
                 
                     
                   Polyvinylpyrrolidone 
                   3 
                 
                     
                   Magnesium stearate 
                   1 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-X. 
     
     
         23 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   mg 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Solid dispersion comprising 
                   156 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   150 
                 
                     
                   Croscarmellose sodium 
                   34 
                 
                     
                   Sodium lauryl sulfate 
                   4 
                 
                     
                   Polyvinylpyrrolidone 
                   15 
                 
                     
                   Magnesium stearate 
                   6 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XI. 
     
     
         24 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   mg 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   150 
                 
                     
                   Solid dispersion comprising 
                   156 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   129 
                 
                     
                   Croscarmellose sodium 
                   30 
                 
                     
                   Sodium lauryl sulfate 
                   4 
                 
                     
                   Polyvinylpyrrolidone 
                   13 
                 
                     
                   Magnesium stearate 
                   5 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XII. 
     
     
         25 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   mg 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Solid dispersion comprising 
                   104 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   128 
                 
                     
                   Croscarmellose sodium 
                   29 
                 
                     
                   Sodium lauryl sulfate 
                   4 
                 
                     
                   Polyvinylpyrrolidone 
                   13 
                 
                     
                   Magnesium stearate 
                   5 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XIII. 
     
     
         26 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   34 
                 
                     
                   Solid dispersion comprising 
                   27 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   25 
                 
                     
                   Croscarmellose sodium 
                   6 
                 
                     
                   Sodium lauryl sulfate 
                   1 
                 
                     
                   Polyvinylpyrrolidone 
                   3 
                 
                     
                   Magnesium stearate 
                   1 
                 
                     
                   Colorant 
                   3 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XIV. 
     
     
         27 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   30 
                 
                     
                   Solid dispersion comprising 
                   31 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   25 
                 
                     
                   Croscarmellose sodium 
                   6 
                 
                     
                   Sodium lauryl sulfate 
                   1 
                 
                     
                   Polyvinylpyrrolidone 
                   3 
                 
                     
                   Magnesium stearate 
                   1 
                 
                     
                   Colorant 
                   3 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XV. 
     
     
         28 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   % by wgt. 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   40 
                 
                     
                   Solid dispersion comprising 
                   21 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   25 
                 
                     
                   Croscarmellose sodium 
                   6 
                 
                     
                   Sodium lauryl sulfate 
                   1 
                 
                     
                   Polyvinylpyrrolidone 
                   3 
                 
                     
                   Magnesium stearate 
                   1 
                 
                     
                   Colorant 
                   3 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XVI. 
     
     
         29 . The tablet  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   mg 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Solid dispersion comprising 
                   156 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Micro crystalline cellulose 
                   150 
                 
                     
                   Croscarmellose sodium 
                   34 
                 
                     
                   Sodium lauryl sulfate 
                   4 
                 
                     
                   Polyvinylpyrrolidone 
                   15 
                 
                     
                   Magnesium stearate 
                   6 
                 
                     
                   Colorant 
                   17 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XVII. 
     
     
         30 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   mg 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Substantially amorphous 
                   125 
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   150 
                 
                     
                   Croscarmellose sodium 
                   34 
                 
                     
                   Sodium lauryl sulfate 
                   4 
                 
                     
                   Polyvinylpyrrolidone 
                   15 
                 
                     
                   Magnesium stearate 
                   6 
                 
                     
                   Colorant 
                   17 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XVIII. 
     
     
         31 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   mg 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   150 
                 
                     
                   Solid dispersion comprising 
                   156 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   129 
                 
                     
                   Croscarmellose sodium 
                   29 
                 
                     
                   Sodium lauryl sulfate 
                   4 
                 
                     
                   Polyvinylpyrrolidone 
                   13 
                 
                     
                   Magnesium stearate 
                   5 
                 
                     
                   Colorant 
                   15 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XIX. 
     
     
         32 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   mg 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Solid dispersion comprising 
                   104 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   128 
                 
                     
                   Croscarmellose sodium 
                   29 
                 
                     
                   Sodium lauryl sulfate 
                   4 
                 
                     
                   Polyvinylpyrrolidone 
                   13 
                 
                     
                   Magnesium stearate 
                   5 
                 
                     
                   Colorant 
                   14 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XX. 
     
     
         33 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                     
                   mg 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Solid dispersion comprising 
                   83 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   128 
                 
                     
                   Croscarmellose sodium 
                   29 
                 
                     
                   Sodium lauryl sulfate 
                   4 
                 
                     
                   Polyvinylpyrrolidone 
                   13 
                 
                     
                   Magnesium stearate 
                   5 
                 
                     
                   Colorant 
                   14 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XXI. 
     
     
         34 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                   Component 
                   % by wgt. 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   20-40 
                 
                     
                   Solid dispersion comprising 
                   30-40 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   20-30 
                 
                     
                   Croscarmellose sodium 
                    1-10 
                 
                     
                   Polyvinylpyrrolidone 
                   1-5 
                 
                     
                   Sodium lauryl sulfate 
                   0.1-1   
                 
                     
                   Magnesium stearate 
                   0.5-1.5 
                 
                     
                     
                 
             
                
                
                
               
               
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XXII. 
     
     
         35 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                   Component 
                   mg/Tablet 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   100 
                 
                     
                   Solid dispersion comprising 
                   156 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   55 
                 
                     
                   Croscarmellose sodium 
                   7 
                 
                     
                   Polyvinylpyrrolidone 
                   11 
                 
                     
                   Sodium lauryl sulfate 
                   3 
                 
                     
                   Total Granules 
                   332 
                 
                     
                   Croscarmellose sodium 
                   18 
                 
                     
                   Microcrystalline cellulose 
                   53 
                 
                     
                   Magnesium stearate 
                   4 
                 
                     
                   Total Tablet 
                   407 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XXIII. 
     
     
         36 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                   Component 
                   mg/Tablet 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   150 
                 
                     
                   Solid dispersion comprising 
                   156 
                 
                     
                   substantially amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline cellulose 
                   65 
                 
                     
                   Croscarmellose sodium 
                   8 
                 
                     
                   Polyvinylpyrrolidone 
                   13 
                 
                     
                   Sodium lauryl sulfate 
                   4 
                 
                     
                   Total Granules 
                   396 
                 
                     
                   Croscarmellose sodium 
                   22 
                 
                     
                   Microcrystalline cellulose 
                   64 
                 
                     
                   Magnesium stearate 
                   5 
                 
                     
                   Total Tablet 
                   487 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XXIV. 
     
     
         37 . The tablet of  claim 19  having the following formulation: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                   Component 
                   mg/Tablet 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   Compound 1 Form I 
                   75 
                 
                     
                   Solid dispersion 
                   156 
                 
                     
                   comprising 
                     
                 
                     
                   substantially 
                     
                 
                     
                   amorphous 
                     
                 
                     
                   Compound 2 
                     
                 
                     
                   Microcrystalline 
                   49 
                 
                     
                   cellulose 
                     
                 
                     
                   Croscarmellose 
                   6 
                 
                     
                   sodium 
                     
                 
                     
                   Polyvinylpyrrolidone 
                   10 
                 
                     
                   Sodium lauryl sulfate 
                   3 
                 
                     
                   Total Granules 
                   299 
                 
                     
                   Croscarmellose 
                   17 
                 
                     
                   sodium 
                     
                 
                     
                   Microcrystalline 
                   48 
                 
                     
                   cellulose 
                     
                 
                     
                   Magnesium stearate 
                   4 
                 
                     
                   Core Tablet 
                   368 
                 
                     
                   Pink Opadry 
                   11 
                 
                     
                   Total Tablet 
                   379 
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
       referred to as PC-XXV. 
     
     
         38 . A method of treating, lessening the severity of, or symptomatically treating cystic fibrosis in a patient comprising administering to the patient an effective amount of the pharmaceutical composition of  claim 1 . 
     
     
         39 . The method of  claim 38 , wherein the pharmaceutical composition has the formulation of any one of PC-I to PC-XXV. 
     
     
         40 . The method of  claim 38 , wherein the patient has a ΔF508 CFTR mutation. 
     
     
         41 . The method of  claim 40 , wherein the patient is homozygous in ΔF508. 
     
     
         42 . The method of  claim 40 , wherein the patient is heterozygous in ΔF508. 
     
     
         43 . A method of preparing a granule comprising wet granulating the following components:
 a. Compound 1 Form I;   b. a solid dispersion comprising substantially amorphous Compound 2;   c. a filler;   d. a disintegrant;   e. a surfactant; and   f. a binder.   
     
     
         44 . A method of preparing a tablet comprising compressing:
 i) a plurality of granular pharmaceutical compositions comprising the following components:
 a. Compound 1 Form I; 
 b. a solid dispersion comprising substantially amorphous Compound 2; 
 c. a filler; 
 d. a disintegrant; 
 e. a surfactant; and 
 f. a binder; 
   ii) a disintegrant;   iii) a filler; and   iv) a lubricant.   
     
     
         45 . A continuous process for preparing a tablet comprising Compound 1 Form I and a solid dispersion comprising substantially amorphous Compound 2 comprising the steps of:
 a) mixing Compound 1 Form I, a solid dispersion comprising substantially amorphous Compound 2, a filler, and a disintegrant in a blender to form a blend;   b) preparing a granulation solution with water, a binder, and a surfactant;   c) feeding the blend from step a) into a continuous twin screw granulator while adding the granulation solution from step b) to produce granules;   d) drying the granules from step c) and milling them;   e) blending the milled granules from step d) with a filler, disintegrant, and lubricant to form a blend; and   f) compressing the blend from step e) into a tablet.   
     
     
         46 . A kit comprising the pharmaceutical composition of  claim 1  and a separate therapeutic agent. 
     
     
         47 . The kit of  claim 46 , wherein the pharmaceutical composition and the therapeutic agent are in separate containers. 
     
     
         48 . The kit of  claim 47 , wherein the containers are bottles, vials, or blister packs, or combination thereof.

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