| abstract |
A composition comprising an effective amount of at least one Factor XII inhibitor (FXII) and at least one C1 inhibitor (C1-INH) for use in treating a contact activation system disorder selected from a thrombotic disorder and a kinin formation-related disorder, wherein the kinin formation-related disorder is selected from hereditary angioedema (HAE), secondary cerebral edema, central nervous system edema, hypotensive shock, and edema during or after contact of blood with an artificial surface, in which the at least one C1-INH is a plasma-derived human C1 esterase inhibitor and/or a recombinant human C1 esterase inhibitor, in which the FXII inhibitor is not C1 INH, and wherein the at least one FXII inhibitor comprises (i) the polypeptide sequence of wild-type infestin-4 (SEQ ID NO: 1), or a polypeptide sequence comprising: (a) SEQ. ID No.: 1 modified to contain 1s 5 amino acid mutations out of N-terminal amino acid positions 2 to 13 of SEQ. ID No.: 1; and/or (b) at least 70%, 80%, 85%, 90%, 95%, 98%, or 99% homology to SEQ. ID No.: 1 and retaining six conserved cysteine residues from SEQ. ID No.: 1; and/or (ii) an anti-FXII antibody wherein the anti-FXII antibody binds to and inhibits the activation and/or activity of FXIIa. |