Practicalities of a reduced volume formulation of a C1-INH concentrate for the treatment of hereditary angioedema: real-life experience

Table 1 Summary of HAE treatments

Drug	Administration	Indications	Adverse events [1, 22]
Plasma-derived C1-INH^a	Intravenous, subcutaneous	Self-administration, acute and prophylaxis^b	Rare: anaphylaxis or thrombosis^c Theoretical: transmission of infectious agent^c Uncommon: injection site reaction, hypersensitivity, nasopharyngitis, dizziness^d
Recombinant human C1-INH	Intravenous	Acute	Uncommon: anaphylaxis
Icatibant	Subcutaneous	Self-administration, acute	Common: local swelling, pain, pruritus at injection site
Ecallantide	Subcutaneous	Acute (only US)	Common: prolonged partial thromboplastin time Uncommon: development of antidrug antibodies, anaphylaxis
Tranexamic acid	Oral, intravenous	Prophylaxis	Common: nausea, vertigo, diarrhea, postural hypotension, fatigue, muscle cramps with increased muscle enzymes Uncommon: thrombosis
Androgens^e	Oral	Prophylaxis	Common: weight gain, virilization, acne, altered libido, muscle pains and cramps, headaches, depression, fatigue, nausea, constipation, menstrual abnormalities, increase in liver enzymes, hypertension, and alterations in lipid profile Uncommon: decreased growth rate in children, masculinization of the female fetus, cholestatic jaundice, peliosis hepatis, and hepatocellular adenoma

^aBerinert^® (IV), Cinryze^® (IV), HAEGARDA^® (SC)
^bBerinert^® approved for: self-administration, acute treatment in adults and pediatrics, short-term prophylaxis in adults and pediatrics (only EU); Cinryze^® approved for: self-administration, prophylaxis in adults and adolescents, acute treatment in adults and pediatrics (only EU); HAEGARDA^® approved (only US) for: self-administration, prophylaxis in adults and adolescents
^cIntravenous administration
^dSubcutaneous administration
^eDanazol, stanozolol and oxandrolone

ISSN: 1710-1492