For a patient with a diagnosis of HAE with normal C1 inhibitor, (HAE nl C1 inh), administration of Icatibant, (Firazyr), did not seem to promptly reverse an attack of tongue swelling. Is there an explanation for this?

Jun 24, 2015

The issue presented raises several interesting points concerning the diagnosis and management of HAE:

  1. To highlight some of the uncertainties regarding the diagnosis of HAE nl C1. It underscores the importance of documenting response to therapy to provide insight as to whether we are dealing with angioedema mediated by bradykinin to guide treatment for the individual patient.
  2. The importance of early treatment to arrest the attacks for all angioedema patients with type I, II and an appropriate diagnosis of nl C1inh.
  3. To reinforce that the on demand therapies arrest the attack but do not reverse the swelling that results from the vascular leakage up to that point. And
  4. The need for a more refined understanding of the efficacy of different therapies for individual patients. This is topic of significant interest to try and ‘marry’ genetic markers for patients and their response to drugs such as icatibant. We are hopeful that funding can be found to further this line of research in the future.

Sandra C

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