Recurrent angioedema
Question:
1/30/2018
A 46 year-old man with h/o oral allergy syndrome hospitalized 12/2017 for throat closing after one day of fever and sore throat treated with Dayquil and Motrin. In ED uvular edema was seen - admitted and treated with steroids, antihistamines, H2 blockers with improvement. Since then has had recurrent episodes of swelling of cheek or lips, eyes - in one episode he had a large urticarial lesion under his arm. All other episodes were only angioedema- usually resolve in 1-2 days with medication. Still having symptoms despite multiple antihistamines and is steroid dependent to prevent attacks. All labs and wu have been neg - HAE wu, including C1q, SPEP, eosinophil count, tryptase, alpha-gal, thyroid studies, RAST to foods. Would you try Xolair or meds for HAE such as icantibant?
Answer:
I asked Dr Bruce Zuraw for his expertise. Some of the information that you provided lacks detail. We assume that you have already considered and ruled out medication and infection (CRS?) induced causes. It is unclear the extent of the HAE evaluation (what tests? any affected family members? any abdominal symptoms?). It is also unclear whether the medications are working or not. How long do the symptoms last if not treated?
It would be helpful to establish whether the angioedema is histamine or bradykinin dependent. The history doesn't sound very bradykinin-like. Response to epinephrine could help sort this out. Lack of response to antihistamines suggests that it is not histamine-dependent but successful management has been observed using 4-fold doses of non-sedating antihistamines in an ongoing manner and prophylactically (not on-demand). Daily montelukast could also be added to the regimen as well. If high dose anti-histamines plus montelukast doesn't help the patient, then omalizumab is a reasonable next step. There are patients who don't have an impressive response to corticosteroids who go on to respond to omalizumab (Faisant et al, J Clin Immunol 2017; 37:80.
We hope that this information helps you.
Jacqueline A. Pongracic, MD, FAAAAI