Angioedema

Asem shadid
Angioedema
 Angioedema is swelling that is similar to hives, but the swelling is
under the skin instead of on the surface.

 It is very similar to urticaria, but urticaria, commonly known as hives,

occurs in the upper dermis. Hives are often called welts. They are a
surface swelling.

 angioedema may affect other parts of body, including respiratory and

gastrointestinal (GI) mucosa. Laryngeal swelling can be life-
threatening.
Types :
The three types of hereditary
angioedema are:

 Type I - decreased levels of C1INH

(85%);
 Type II - normal levels, but decreased
function of C1INH (15%);
 Type III - no detectable abnormality in
C1INH, occurs in an X-linked dominant
fashion and therefore mainly affects
women; it can be exacerbated by
pregnancy and use of hormonal
contraception (exact frequency uncertain).
Pathophysiology :
 Angioedema is a result of the fast onset of an increase in
local vascular permeability in subcutaneous or submucosal
tissue. Histamine and bradykinin are the most recognized
vasoactive mediators known to be .

 Other vasoactive mediators are, at least in part, involved in

the pathogenesis of various types of angioedema.
Leukotrienes, for example, may play an important role in the
onset of angioedema that is induced by nonsteroidal anti-
inflammatory drugs (NSAIDs) .

 Thus, factors influencing histamine release, bradykinin

metabolism, and endothelial cell function or permeability may
directly or indirectly regulate the process of angioedema.
Con . Histamine- and bradykinin-mediated
angioedema :

 For histamine-mediated angioedema (histaminergic

angioedema), mast cells and basophils are the primary
sources of histamine.

 The activation of mast cells or basophils with

subsequent histamine release may be either mediated
or unmediated by immunoglobulin E (IgE).

 IgE-mediated mast cell activation and degranulation,

key elements of an allergic reaction, often manifest as
urticaria and angioedema.

 Type I hypersensitivity reactions, such as food or drug

allergies, are typically IgE-mediated.
Etiology
 More than 40% of chronic angioedema is idiopathic. Trauma,
surgical procedures, and stress are common nonspecific
triggers for angioedema attacks.
 Angioedemas with identifiable etiologies include those
caused by the following:
 Hypersensitivity (eg, food, drugs, or insect stings)
 Physical stimuli (eg, cold or vibrations)
 Autoimmune disease or infection
 ACE inhibitors
 NSAIDs
 C1-INH deficiency (hereditary and acquired)
Exams and Tests
 The health care provider will look at your skin and ask
you if you have been exposed to any irritating
substances.
 A physical exam might reveal abnormal sounds
(stridor) when you breathe in if your throat is affected.
 Blood tests or allergy testing may be ordered.

 Testing for and Diagnosing Hereditary Angioedema

 Blood tests can check the C1 inhibitor level and
function.
 Since Cl inhibitor is part of the complement system,
blood tests for complement components C4 and C2
may also help diagnose HAE.
 What Are the Symptoms of HAE?
 The symptoms of HAE are
grouped by the area of
inflammation. Swelling may affect  Symptoms of throat
the abdomen, face, throat, limbs, inflammation include:
and intestines. According to the
Genetic and Rare Diseases
Information Center, an episode of  hoarse voice
swelling may last for up to four  difficulty swallowing
days (GRDIC).  difficulty breathing

 Symptoms of intestinal swelling  Other symptoms include

include: swelling in the:

 Severe abdominal pain and  legs

cramping
 arm
 dehydration
 eyes
 diarrhea
 throat
 shock (in severe cases)
 tongue
 Management
 Allergic
 n allergic angioedema, avoidance of the allergen and
use of antihistamines may prevent future attacks.
 Cetirizine is a commonly prescribed antihistamine for
angioedema.

 Some patients have reported success with the

combination of a nightly low dose of cetirizine to
moderate the frequency and severity of attacks,
followed by a much higher dose when an attack does
appear.
 Severe angioedema cases may require desensitization
to the putative allergen, as mortality can occur.
 Chronic cases require steroid therapy, which generally
leads to a good response.
 In cases where allergic attack is progressing towards
airway obstruction, epinephrine may be life-saving.
Management
 Drug induction
 ACE inhibitors can induce angioedema. ACE inhibitors
block the enzyme ACE so it can no longer degrade
bradykinin; thus, bradykinin accumulates and causes
angioedema.

 This complication appears more common in African-

Americans.[14] In people with ACE inhibitor
angioedema, the drug needs to be discontinued and an
alternative treatment needs to be found, such as an
angiotensin II receptor blocker (ARB)\ which has a
similar mechanism but does not affect bradykinin.

 However, this is controversial, as small studies have

shown some patients with ACE inhibitor angioedema
can develop it with ARBs, as well.
Management
 Hereditary
 In hereditary angioedema, specific stimuli that have
previously led to attacks may need to be avoided in the
future.

 It does not respond to antihistamines, corticosteroids, or

epinephrine. Acute treatment consists of C1-INH
concentrate from donor blood, which must be
administered intravenously.

 In an emergency, fresh frozen blood plasma, which

also contains C1-INH, can also be used.

 However, in most European countries, C1-INH

concentrate is only available to patients who are
participating in special programmes.
Management
 Acquired
 In acquired angioedema, HAE types I and II, and
nonhistaminergic angioedema, antifibrinolytics
such as tranexamic acid or ε-aminocaproic acid
may be effective.

 Cinnarizine may also be useful because it blocks

the activation of C4 and can be used in patients
with liver disease, while androgens cannot
Treatment
 Several types of medication and therapies can be used
to treat HAE.
 Epinephrine is used in life-threatening situations to
keep the airway open. It tightens blood vessels and
relaxes muscles in the throat. Epinephrine is more
effective than antihistamines—antihistamines have not
been proven to be as effective in treatment of HAE. It
also works more quickly, so is better in emergency
situations.
 C1-inhibitors are a very effective way to treat HAE.
However, they are not always available.
 Fresh frozen plasma might be beneficial in treating the
symptoms of HAE.
 Attenuated androgens (such as danazol) may be able
to increase production of C1 inhibitor in people with
type I HAE.
 Treatment
 Mild symptoms may not need
treatment. Moderate to severe
symptoms may need to be
treated. Breathing difficulty is an  Antihistamines
emergency condition.
 People with angioedema
should:
 Avoid any known allergen or
trigger that causes their
symptoms
 Avoid any medicines, herbs,
or supplements that are not
prescribed by a health care
provider
 Cool compresses or soaks
can relieve pain.
 Medications used to treat
angioedema include:
 Anti-inflammatory medicines
(corticosteroids)
 Epinephrine shots (people
with a history of severe
symptoms can carry these
with them)
 Inhaler medicines that help
open up the airways
 If the person has trouble
breathing, seek immediate
medical help. A severe, life-
threatening airway blockage
may occur if the throat
swells.
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