Table 1 Ad hoc C1-INH-HAE severity score
Severity score | Criteria |
|---|---|
Asymptomatic | No angioedema episodes and no long-term prophylactic treatment |
Mild | No life-threatening angioedema episodes and no long-term prophylactic treatment and ≤ 3 episodes/last 6 mos |
Moderate | No life-threatening angioedema episodes and ≤ 6 episodes/last 6 mos. with long-term prophylactic treatment (except maintenance treatment with pdC1-INH) or > 4–12 episodes/last 6 mos. without long-term prophylactic treatment |
Severe | Life-threatening angioedema episodes and/or 6 episodes/last 6 mos. with long-term prophylactic treatment and/or Maintenance treatment with pdC1-INH and/or > 12 episodes/last 6 mos. without long-term prophylactic treatment |