Treatment of Acquired C1 Inhibitor Deficiency in AAE-C1-INH with Fewer Than 2 Attacks per Week

Clinical Scenario

This protocol addresses patients with a confirmed diagnosis of acquired angioedema secondary to C1 esterase inhibitor deficiency (AAE-C1-INH) who experience fewer than 2 attacks per week. MDT and relevant specialist input is required to diagnose and treat the underlying cause of AAE before additional prophylactic treatment is considered.

Treatment Approach

When prophylaxis is required in this setting, oral antifibrinolytic therapy has a role. The full treatment algorithm — including additional prophylactic options, clinical criteria that guide their selection, and considerations for patients already established on existing therapies — is available in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

Patient with a diagnosis of AAE-C1-INH
MDT and relevant specialist input to diagnose and treat the underlying cause of AAE. If additional treatment required:
<2 attacks per week
Tranexamic acid
Some adult patients are treated with androgens as oral prophylactic treatment.
Where existing patients are established on androgen therapy, this may continue if considered clinically appropriate; if established patients do cease treatment with androgen therapy then review the need for any prophylaxis.

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