Hereditary angioedema

1. Hereditary angiedema
By.. Doctor hosam Maarouf Alhussin

2. Hereditary
angioedema
(HAE)
is an autosomal dominant
disease caused by either a lack of
C1-inhibitor protein or
dysfunctional C1-inhibitor
protein. HAE manifests with
symptoms related to
angioedema of the upper airway,
skin, and/ or gastrointestinal
tract.

3. What are the symptoms of
hereditary angioedema?
• These symptoms may include:
• painless, non-itchy rash.
• tingling skin.
• skin tightness.
• fatigue.
• irritability.
• sudden mood changes.
• anxiety.

8. What is the mechanism of
hereditary angioedema?
loss of control over the plasma contact
system or kallikrein-kinin system, which
results in unrestrained bradykinin
generation or signaling

10. Is angioedema bradykinin vs
histamine?
Most cases of angioedema are attributable to
the vasoactive mediators histamine and
bradykinin. Histamine-mediated (allergic)
angioedema occurs through a type I
hypersensitivity reaction, whereas bradykinin-
mediated (non-allergic) angioedema is
iatrogenic or hereditary in origin.

11. What are the 4 types of
angioedema?
Acute allergic angioedema. Non-
allergic drug reaction. Idiopathic
angioedema. Hereditary angioedema

12. What tests are used to diagnose
angioedema?

13. What is the first line treatment for
hereditary angioedema?in the past
Hereditary angioedema (HAE) is generally
refractory to treatment with
catecholamines, antihistamines, and
steroids. Anabolic steroids, such as danazol,
may be used for the acute phase of an attack
of HAE.

14. Now
ICATIBANT (GENERIC) is a bradykinin B2
receptor antagonist indicated for treatment of
acute attacks of Hereditary Angioedema (HAE)
in adults 18 years of age and older. Generic
Icatibant is delivered by subcutaneous injection
and is approved for self-administration.
Multiple manufacturers offer Generic Icatibant.