Hereditary C1 Inhibitor Deficiency When First-Line Therapy Is Unavailable or Has Not Achieved Resolution

Acute attacks of hereditary C1 inhibitor deficiency are treated on-demand, as early as possible, with intravenous C1 inhibitor, ecallantide, or icatibant. When those first-line agents are not available, or when they have not achieved resolution of angioedema symptoms and a shorter total attack duration, a further treatment step is indicated.

First-line failure condition

On-demand therapy with intravenous C1 inhibitor, ecallantide, or icatibant did not achieve resolution of angioedema symptoms and shorter total attack duration, or these agents were not accessible at the time of the attack.

Next-step approach

The protocol calls for a specific plasma-derived product as an alternative acute attack intervention — the complete agent, administration details, and clinical algorithm are available in the full structured regimen.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1111/all.15214
Where these first-line therapies are not available, attacks should be treated with solvent detergent-treated plasma (SDP).

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