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Is Angioedema Hereditary?
My question is should I pursue the possibility of Hereditary Angioedema if I have had SEVERE facial swelling to aspirin (two episodes) , several systemic bee stings reactions for which adrenaline was given, and a presumed post operative angioedemic abdomen. I have had lesser self-limited angioedema to IVP dye and some unknown causes. I have son who had facial angioedema and Stevens Johnson reaction to penicillin with severe blisters/burns to his fingers. And I have a brother who was seconds away from a surgical airway from an unknown cause when he arrived at the ER. Obviously I suspect this may be hereditary. My question is , is knowing we have that form or not important? Thank you.
Thu, 11 Feb 2021
Hi,
The family history and personal history given by you in the query is suggestive of hereditary angioedema, an autosomal dominant condition. In this condition, uncontrolled release of bradykinin (an inflammatory mediator present in body) results in tissue edema during an attack. The diagnosis can be made by evaluating presenting symptoms, family history, and investigations like low plasma levels of C1 inhibitor protein.
Treatment involves use of IV C1 inhibitor concentrate or fresh frozen plasma during an acute attack. Usually, patients do not respond to adrenaline, steroids and antihistamines. In some cases, prophylactic use of anabolic steroid like danazol may be helpful.
Take care. Hope I have answered your question. Let me know if I can assist you further.
Regards,
Dr. Ayesha Shareef, General & Family Physician
The family history and personal history given by you in the query is suggestive of hereditary angioedema, an autosomal dominant condition. In this condition, uncontrolled release of bradykinin (an inflammatory mediator present in body) results in tissue edema during an attack. The diagnosis can be made by evaluating presenting symptoms, family history, and investigations like low plasma levels of C1 inhibitor protein.
Treatment involves use of IV C1 inhibitor concentrate or fresh frozen plasma during an acute attack. Usually, patients do not respond to adrenaline, steroids and antihistamines. In some cases, prophylactic use of anabolic steroid like danazol may be helpful.
Take care. Hope I have answered your question. Let me know if I can assist you further.
Regards,
Dr. Ayesha Shareef, General & Family Physician
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