Method of Treating Myasthenia Gravis

Abstract

The invention relates to the use of agents that bind the complement protein C5 in the treatment of diseases associated with inappropriate complement activation and in particular in the treatment of myasthenia gravis.

1 - 20 . (canceled) 
     
     
         21 . A method of treating myasthenia gravis comprising administering to a human subject in need thereof a therapeutically effective amount of an agent that binds complement C5 protein, wherein the agent is a protein comprising an amino acid sequence having at least 90% sequence identity to amino acids 19 to 168 of SEQ ID NO: 2. 
     
     
         22 . The method according to  claim 21 , wherein the agent binds complement C5 protein with an IC50 of less than 0.2 mg/ml. 
     
     
         23 . The method according to  claim 21 , wherein the agent is derived from a haematophagous arthropod. 
     
     
         24 . The method according to  claim 21 , wherein the therapeutically effective amount is a dose from 1 mg/kg to 15 mg/kg. 
     
     
         25 . The method according to  claim 21 , wherein the agent is administered as part of a treatment regimen also involving the administration of a further drug for the treatment of myasthenia gravis. 
     
     
         26 . The method according to  claim 25 , wherein the further drug is an anticholinesterase agent, neostigmine, or pyridostigmine, or an immunosuppressive drug, prednisone, cyclosporine, or azathioprine. 
     
     
         27 . The method according to  claim 25 , wherein the agent is administered simultaneously with the further drug. 
     
     
         28 . The method according to  claim 25 , wherein the agent is administered sequentially with the further drug. 
     
     
         29 . The method according to  claim 25 , wherein the agent is administered separately from the further drug. 
     
     
         30 . The method according to  claim 21 , wherein the myasthenia gravis is mild myasthenia gravis. 
     
     
         31 . The method according to  claim 21 , wherein the myasthenia gravis is severe myasthenia gravis. 
     
     
         32 . The method according to  claim 21 , wherein the therapeutically effective amount is a dose from 1 mg/kg to 10 mg/kg. 
     
     
         33 . The method according to  claim 21 , wherein the agent is a protein comprising an amino acid sequence having at least 95% sequence identity to amino acids 19 to 168 of SEQ ID NO: 2. 
     
     
         34 . The method according to  claim 21 , wherein the agent is a protein comprising an amino acid sequence having at least 98% sequence identity to amino acids 19 to 168 of SEQ ID NO: 2. 
     
     
         35 . A method of treating myasthenia gravis comprising administering to a human subject in need thereof a therapeutically effective amount of an agent that binds complement C5 protein, wherein the agent is a protein comprising at least a fragment of SEQ ID NO: 2, wherein said fragment comprises six cysteine residues that are spaced relative to each other at a distance of 32 amino acids apart, 62 amino acids apart, 28 amino acids apart, 1 amino acid apart, and 21 amino acids apart, respectively, as arranged from the amino terminus to the carboxyl terminus of SEQ ID NO: 2, wherein said fragment inhibits cleavage of C5 by classical and alternative C5 convertases. 
     
     
         36 . The method according to  claim 36 , wherein the agent is administered on a daily basis or every two or three days. 
     
     
         37 . The method according to  claim 36 , wherein the agent is a protein comprising amino acids 19 to 168 of SEQ ID NO: 2. 
     
     
         38 . The method according to  claim 36 , wherein the agent is a protein consisting of amino acids 19 to 168 of SEQ ID NO: 2. 
     
     
         39 . A method of treating myasthenia gravis comprising administering to a human subject in need thereof a therapeutically effective amount of an agent that binds complement C5 protein, wherein the agent is a nucleic acid encoding a protein comprising at least a fragment of SEQ ID NO: 2, wherein said fragment comprises six cysteine residues that are spaced relative to each other at a distance of 32 amino acids apart, 62 amino acids apart, 28 amino acids apart, 1 amino acid apart, and 21 amino acids apart, respectively, as arranged from the amino terminus to the carboxyl terminus of SEQ ID NO: 2, wherein said fragment inhibits cleavage of C5 by classical and alternative C5 convertases.