Table 2. HAE-Specific Agents
Drug Name
(Brand)
FDA
Indications Dosage Mechanism
Anticipated
Potential Side
Effects
Plasma-derived
nanofiltered
C1INH
(Cinryze
®
)
Long-term
prophylaxis
1000 units IV
q3-4d
Inhibits plasma
kallikrein,
coagulation
factors XIIa
and XIa, C1s,
C1r, MASP-1,
MASP-2, and
plasmin
Rare: risk of
anaphylaxis
eoretical:
transmission
of infectious
agent
Plasma-derived
nanofiltered
C1INH
(Berinert
®
P)
Acute attacks 20 units per
kg IV
Inhibits plasma
kallikrein,
coagulation
factors XIIa
and XIa, C1s,
C1r, MASP-1,
MASP-2, and
plasmin
Rare: risk of
anaphylaxis
eoretical:
transmission
of infectious
agent
Ecallantide
(Kalbitor
®
)
Acute attacks 30 mg subcuta-
neous
(administered
as 3 injections
of 1 mL each)
Inhibits plasma
kallikrein
Uncommon:
anti-drug anti-
bodies, risk of
anaphylaxis
Icatibant
(Firazyr
®
)
Acute attacks 30 mg subcuta-
neous
Bradykinin
B2 receptor
antagonist
Common:
injection site
reactions
Recombinant-
human C1INH
(Rhucin
®
)
Acute attacks
(pending )
50-100 units
per kg IV
Inhibits plasma
kallikrein,
coagulation
factors XIIa
and XIa, C1s,
C1r, MASP-1,
MASP-2, and
plasmin
Uncommon:
risk of anaphy-
laxis in rabbit-
sensitized
individuals
Adapted from Zuraw, BL. N Engl J Med. 2008;359(10):1027-36.
