Table 2.
Specific risks and adverse events of products for the treatment of HAE-C1-INH
| Active ingredient/trade name | Risks and adverse events |
|---|---|
| Human pdC1-INH concentrates | |
| Berinert® | A theoretical risk of pathogen transmission is associated with all plasma products. No such transmissions have thus far been described. In this respect, the product can be judged to be safe. It was speculated that long-term use of Berinert may be associated with an increased frequency of HAE-C1-INH attacks [2]. |
| Cinryze® | A theoretical risk of pathogen transmission is associated with all plasma products. No such transmissions have thus far been described. In this respect, the product can be judged to be safe. |
| Recombinant human C1-INH concentrate | |
| Ruconest® | Several patients developed antibodies to rabbit antigens (from dander and hair, not from the C1-INH); allergic reactions were observed rarely. |
| Kallikrein and bradykinin receptor antagonists | |
| Icatibant/Firazyr® | From the theoretical perspective, caution is advised in patients with ischemic heart disease, unstable angina pectoris, and in the first weeks following a stroke. Clinically relevant problems in this regard have not been observed to date. |
| Ecallantide/Kalbitor™ | Worth mentioning is the risk of anaphylactic reactions (frequency according to the boxed warning in the USA full prescribing information, 3.9 % [21]). |
| Attenuated androgens | |
| Danazol/Danatrol® | The most common adverse effects are virilization, weight gain, menstrual irregularities, depression/aggression, myalgia, and acne. Adverse effects such as hypercholesterolemia, hypertension, erythrocytosis, and hepatic tumors mandate the need for regular medical checkups. Furthermore, in children, growth disorders and premature closure of the epiphyseal cartilage are conceivable and have not been sufficiently analyzed in clinical studies. |
| Danazol/Danokrin® | |
| Danazol/Danocrine™ | |
| Stanozolol/Winstrol™ | |
| Antifibrinolytics | |
| Tranexamic acid/Cyklokapron® | The most common adverse effects are dose-dependent gastrointestinal symptoms (nausea, vomiting, and diarrhea). |