Hereditary C1 inhibitor deficiency
ICD-10 D84.1 · ICD-11 4A00.14
On-Demand Treatment of Acute Attacks in Hereditary C1 Inhibitor Deficiency
Clinical Scenario
Hereditary C1 inhibitor deficiency causes recurrent angioedema attacks of unpredictable onset and severity. Managing each acute episode promptly is central to care — early intervention has direct consequences for how quickly and completely the attack resolves.
Treatment Goals
Resolution of angioedema symptoms
Shorter total attack duration
Treatment Approach (partial)
References
DOI: 10.1111/all.15214
- We recommend that attacks are treated with either intravenous C1 inhibitor, ecallantide or icatibant.
- For HAE-1/2, icatibant, ecallantide and intravenous C1-INH are the recommended on-demand treatments of choice.
- We recommend that attacks are treated as early as possible.
- Early treatment is associated with a shorter time to resolution of symptoms and shorter total attack duration regardless of attack severity.