US2019038623A1PendingUtilityA1

Therapeutic regimens for treatment of paroxysmal nocturnal hemoglobinuria

Assignee: ACHILLION PHARMACEUTICALS INCPriority: Aug 2, 2017Filed: Aug 2, 2018Published: Feb 7, 2019
Est. expiryAug 2, 2037(~11 yrs left)· nominal 20-yr term from priority
C07K 2317/76A61P 7/00C07K 16/18A61K 2039/505A61K 39/3955A61K 31/506
51
PatentIndex Score
0
Cited by
0
References
0
Claims

Abstract

Provided herein are methods for treating a subject with PNH comprising administering to a subject a therapeutically effective amount of complement component C5 (C5) inhibitor, complement component C3 (C3) inhibitor, or complement factor B (CFB) inhibitor in combination with a therapeutically effective amount of small molecule complement factor D (CFD) inhibitor of Formula I or Formula II, or a pharmaceutically acceptable salt thereof.

Claims

exact text as granted — not AI-modified
1 . A method for treating paroxysmal nocturnal hemoglobinuria (PNH) in a subject in need thereof comprising administering to the subject a therapeutically effective amount of a complement 5 (C5) inhibitor in combination with a therapeutically effective amount of a complement factor D (CFD) inhibitor;
 wherein the CFD inhibitor is selected from a compound of Formula I or Formula II:   
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof; 
         wherein: 
         X is selected from N and CH; 
         R 1  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 2  is selected from hydrogen and C 1 -C 3  alkyl; 
         R 3  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 4  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; and 
         R 5  is selected from hydrogen, C 1 -C 3  alkyl, halogen, and cyano. 
       
     
     
         2 . The method of  claim 1 , wherein the CFD inhibitor is selected from the group consisting of a compound of formula: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
     
     
         3 . The method of  claim 1 , wherein the CFD inhibitor is selected from the group consisting of a compound of formula: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
     
     
         4 . The method of  claim 1 , wherein the CFD inhibitor is a compound of the formula: 
       
         
           
           
               
               
           
         
       
     
     
         5 . The method of  claim 1 , wherein the C5 inhibitor is eculizamab. 
     
     
         6 . The method of  claim 1 , wherein the C5 inhibitor is selected from the group consisting of a recombinant human minibody, coversin, Tesidolumab/LFG316, ARC-1905, RA101348, RA101495, SOBI002, ARC1005, a SOMAmer for C5, SSL7, MEDI7814, aurin tricarboxylic acid, an aurin tricarboxylic acid derivative, RG6107/SKY59, ALXN1210, ALXN5500, TT30, ABP959, Anti-05 siRNA, Erdigna, avacincaptad pegol/Zimura®, SOBI005, ISU305, and REGN3918. 
     
     
         7 . A method for treating paroxysmal nocturnal hemoglobinuria (PNH) in a subject in need thereof comprising administering to the subject a therapeutically effective amount of a complement factor D (CFD) inhibitor selected from a compound of Formula I or Formula 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof; 
         wherein: 
         X is selected from N and CH; 
         R 1  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 2  is selected from hydrogen and C 1 -C 3  alkyl; 
         R 3  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 4  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 5  is selected from hydrogen, C 1 -C 3  alkyl, halogen, and cyano; 
         wherein the subject at the time of administration of the CFD inhibitor has been receiving or is currently receiving a therapeutic regimen comprising the administration of a complement 5 (C5) inhibitor; and, 
         wherein the subject is experiencing extravascular hemolysis. 
       
     
     
         8 . The method of  claim 7 , wherein the CFD inhibitor is selected from the group consisting of a compound of formula: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
     
     
         9 . The method of  claim 7 , wherein the CFD inhibitor is selected from the group consisting of a compound of formula: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
     
     
         10 . The method of  claim 7 , wherein the CFD inhibitor is a compound of the formula: 
       
         
           
           
               
               
           
         
       
     
     
         11 . The method of  claim 7 , wherein the C5 inhibitor is eculizamab. 
     
     
         12 . The method of  claim 7 , wherein the C5 inhibitor is selected from the group consisting of a recombinant human minibody, coversin, Tesidolumab/LFG316, ARC-1905, RA101348, RA101495, SOBI002, ARC1005, a SOMAmer for C5, SSL7, MEDI7814, aurin tricarboxylic acid, an aurin tricarboxylic acid derivative, RG6107/SKY59, ALXN1210, ALXN5500, TT30, ABP959, Anti-05 siRNA, Erdigna, avacincaptad pegol/Zimura®, SOBI005, ISU305, and REGN3918. 
     
     
         13 . The method of  claim 7 , wherein the subject has a hemoglobin level of less than about 10 g/dL at the time of administration of the CFD inhibitor. 
     
     
         14 . The method of  claim 7 , wherein the subject has an LDH level within a normal range. 
     
     
         15 . The method of  claim 7 , wherein the subject has a LDH level of less than 250 U/L at the time of administration of the CFD inhibitor. 
     
     
         16 . The method of  claim 7 , wherein the subject has received one or more blood transfusions within the twelve months prior to administration of the CFD inhibitor. 
     
     
         17 . The method of  claim 7 , wherein the subject has been on a C5 therapeutic regimen for at least three months prior to administration of the CFD inhibitor. 
     
     
         18 . The method of  claim 7 , wherein upon administration of the CFD inhibitor, the C5 inhibitor also continues to be administered. 
     
     
         19 . The method of  claim 7 , wherein upon administration of the CFD inhibitor, the C5 inhibitor is no longer administered. 
     
     
         20 . A method for treating paroxysmal nocturnal hemoglobinuria (PNH) in a subject in need thereof comprising administering to the subject a therapeutically effective amount of a complement factor D (CFD) inhibitor selected from a compound of Formula I or Formula 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof; 
         wherein: 
         X is selected from N and CH; 
         R 1  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 2  is selected from hydrogen and C 1 -C 3  alkyl; 
         R 3  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 4  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 5  is selected from hydrogen, C 1 -C 3  alkyl, halogen, and cyano; 
         wherein the subject at the time of administration of the CFD inhibitor has been receiving or is currently receiving a therapeutic regimen comprising the administration of a complement 5 (C5) inhibitor; and 
         wherein the subject is experiencing residual intravascular hemolysis. 
       
     
     
         21 . The method of  claim 20 , wherein the CFD inhibitor is selected from the group consisting of a compound of formula: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
     
     
         22 . The method of  claim 20 , wherein the CFD inhibitor is selected from the group consisting of a compound of formula: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
     
     
         23 . The method of  claim 20 , wherein the CFD inhibitor is a compound of the formula: 
       
         
           
           
               
               
           
         
       
     
     
         24 . The method of  claim 20 , wherein the C5 inhibitor is eculizamab. 
     
     
         25 . The method of  claim 20 , wherein the C5 inhibitor is selected from the group consisting of a recombinant human minibody, coversin, Tesidolumab/LFG316, ARC-1905, RA101348, RA101495, SOBI002, ARC1005, a SOMAmer for C5, SSL7, MEDI7814, aurin tricarboxylic acid, an aurin tricarboxylic acid derivative, RG6107/SKY59, ALXN1210, ALXN5500, TT30, ABP959, Anti-05 siRNA, Erdigna, avacincaptad pegol/Zimura®, SOBI005, ISU305, and REGN3918. 
     
     
         26 . The method of  claim 20 , wherein the subject has a hemoglobin level of less than about 10 g/dL at the time of administration of the CFD inhibitor. 
     
     
         27 . The method of  claim 20 , wherein the subject has a LDH level greater than the upper level of normal. 
     
     
         28 . The method of  claim 20 , wherein the subject has a LDH level of greater than 250 U/L at the time of administration of the CFD inhibitor. 
     
     
         29 . The method of  claim 20 , wherein the subject has received one or more blood transfusions within the twelve months prior to administration of the CFD inhibitor. 
     
     
         30 . The method of  claim 20 , wherein the subject has been on a C5 therapeutic regimen for at least three months prior to administration of the CFD inhibitor. 
     
     
         31 . The method of  claim 20 , wherein upon administration of the CFD inhibitor, the C5 inhibitor also continues to be administered. 
     
     
         32 . The method of  claim 20 , wherein upon administration of the CFD inhibitor the C5 inhibitor is no longer administered. 
     
     
         33 . A method for treating paroxysmal nocturnal hemoglobinuria (PNH) in a subject in need thereof comprising administering to the subject a therapeutically effective amount of a complement factor D (CFD) inhibitor selected from a compound of Formula I or Formula 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof; 
         wherein: 
         X is selected from N and CH; 
         R 1  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 2  is selected from hydrogen and C 1 -C 3  alkyl; 
         R 3  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 4  is selected from hydrogen, C 1 -C 3  alkyl, and halogen; 
         R 5  is selected from hydrogen, C 1 -C 3  alkyl, halogen, and cyano; 
         wherein the subject at the time of administration of the CFD inhibitor has been receiving or is currently receiving a therapeutic regimen comprising the administration of a complement 5 (C5) inhibitor; and 
         wherein the subject has a hemoglobin level of less than about 10 g/dL. 
       
     
     
         34 . The method of  claim 33 , wherein the CFD inhibitor is selected from a compound of formula: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
     
     
         35 . The method of  claim 33 , wherein the CFD inhibitor is selected from a compound of formula: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
     
     
         36 . The method of  claim 33 , wherein the CFD inhibitor is a compound of the formula: 
       
         
           
           
               
               
           
         
       
     
     
         37 . The method of  claim 33 , wherein the C5 inhibitor is eculizamab. 
     
     
         38 . The method of  claim 33 , wherein the C5 inhibitor is selected from a recombinant human minibody, coversin, Tesidolumab/LFG316, ARC-1905, RA101348, RA101495, SOBI002, ARC1005, a SOMAmer for C5, SSL7, MEDI7814, aurin tricarboxylic acid, an aurin tricarboxylic acid derivative, RG6107/SKY59, ALXN1210, ALXN5500, TT30, ABP959, Anti-C5 siRNA, Erdigna, avacincaptad pegol/Zimura®, SOBI005, ISU305, and REGN3918. 
     
     
         39 . The method of  claim 33 , wherein the subject has a hemoglobin level of less than about 8 g/dL at the time of administration of the CFD inhibitor. 
     
     
         40 . The method of  claim 33 , wherein the subject has a LDH level greater than the upper level of normal. 
     
     
         41 . The method of  claim 33 , wherein the subject has a LDH level of greater than about 250 U/L at the time of administration of the CFD inhibitor. 
     
     
         42 . The method of  claim 33 , wherein the subject has a LDH level within a normal range. 
     
     
         43 . The method of  claim 33 , wherein the subject has a LDH level of less than about 250 U/L at the time of administration of the CFD inhibitor. 
     
     
         44 . The method of  claim 33 , wherein the subject has received one or more blood transfusions within the twelve months prior to administration of the CFD inhibitor. 
     
     
         45 . The method of  claim 33 , wherein the subject has been on a C5 therapeutic regimen for at least three months prior to administration of the CFD inhibitor. 
     
     
         46 . The method of  claim 33 , wherein upon administration of the CFD inhibitor, the C5 inhibitor also continues to be administered. 
     
     
         47 . The method of  claim 33 , wherein upon administration of the CFD inhibitor the C5 inhibitor is no longer administered.

Join the waitlist — get patent alerts

Track US2019038623A1 — get alerts on status changes and closely related new filings.

We store only your email — no account needed. See our privacy policy.