Acquired angioedema secondary to C1 esterase inhibitor deficiency (AAE-C1-INH) can escalate rapidly. When an acute attack is clinically significant — demanding admission or injected treatment — timely, protocol-driven management is critical.

This protocol targets patients with confirmed AAE-C1-INH whose current attack qualifies as clinically significant on risk assessment: either potentially life-threatening because it involves the head or neck, or causing sufficient pain or disability that the patient cannot continue normal activities — and for whom admission or injected treatment is indicated.

First-line treatment approach

Initial management centres on specific targeted pharmacological agents. The protocol addresses which product type to consider first and, where licensed recombinant options are available, when these should be preferred over plasma-derived products for acute attack treatment.

Full agent selection criteria, sequencing, and additional detail are available in the complete protocol below.

References

Patient with a diagnosis of AAE-C1-INH with a clinically significant attack that requires admission/injected treatment on clinical and risk assessment
A clinically significant attack as defined by i) potentially life threatening because it affects the head or neck or ii) causes pain or disability such that the patient cannot continue their normal activities.
Icatibant
Intravenous C1-esterase inhibitor
In appropriate cases and where available, licensed recombinant products should be considered in preference to plasma-derived products in the treatment of acute attacks.

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Treatment of Acquired C1 Inhibitor Deficiency with Clinically Significant Acute Attack Requiring Admission

First-line treatment approach

References