Angioedema

Done By: Reem Alyahya

Tutor:Dr.Dalia Ashour

Group: 6

Introduction

• Name: Maha

• Age: 21 years

• Symptoms: very tight throat , noisy breathing, difficulty swallowing , horsey voice, swollen eyes and lips, anxiety.

• The complaint arose after eating a Magnum almond ice cream.

Objectives: The definition of

angioedema

Types of angioedema

The causes of angioedema

The Pathophysiology of angioedema

The risk factors of angioedema

The signs and symptoms of angioedema

The diagnosis and the differential diagnosis of angioedema

The treatment of angioedema

The prognosis of angioedema 

Definition Of Angioedema

• Angioedema or Quincke's edema is the rapid swelling (edema) of the dermis, subcutaneous tissue,  mucosa and submucosal tissues. It is very similar to urticaria, but urticaria, commonly known as hives, occurs in the upper dermis.

Types Of Angioedema

1. Acute or allergic angioedema – the swelling is caused by an allergic reaction, such as a reaction to peanuts.

2. idiopathic angioedema – there is no known cause for the symptoms of swelling .

• 3. drug-induced angioedema – the swelling is a side effect of certain medications.

• 4. hereditary angioedema – the swelling is caused by ‘faulty’ genes that are inherited from a person’s parents.

Types Of Angioedema

• Or Angioedema is classified as either hereditary or acquired.

1. Acquired angioedema can be immunologic, nonimmunologic, or idiopathic.  It is usually caused by allergy and occurs together with other allergic symptoms and urticaria. It can also occur as a side effect to certain medications, particularly ACE inhibitors.

• Hereditary angioedema (HAE): exists in three forms, all of which are caused by a genetic mutation inherited in an autosomal dominant form. They are distinguished by the underlying genetic abnormality.

Types I•mutations in the SERPING1gene•Lead to diminished levels of the C1inhibitor protein

Types II•mutations in the SERPING1gene•Lead to dysfunctional forms of C1-inhibitor protein

Types III•mutations in the F12 gene, •which encodes the coagulation protein factor XII

All forms of HAE lead to abnormal

activation of the complement system,

and all forms can cause swelling

elsewhere in the body, such as

the digestive tract.

Causes of Angioedema

• Animal dander

• Exposure to water, sunlight, cold or heat

• Foods (such as berries, shellfish, fish, nuts, eggs, and milk)

• Insect bites

• Medicines (drug allergy), such as antibiotics (penicillin), nonsteroidal anti-inflammatory drugs (NSAIDs), and blood pressure medicines.

• Pollen

•  infections or with other illnesses (including autoimmune, and leukemia and lymphoma).

• Family history ( HAE )

Pathophysiology

• Angioedema is swelling due to increased vascular permeability and extravasation of intravascular fluid. Known mediators of increased vascular permeability include the following:

1. Mast cell–derived mediators (e.g. histamine).

2. Bradykinin, a peptide containing nine amino acid residues produced from α2-globulin by the enzyme kallikrein. Bradykinin is a potent vasodilator, and complement-derived mediators.

Allergic angioedema: (just

like anaphylaxis):

Hereditary angioedema:

• Hereditary angioedema (HAE) is due to mutations within the C1-INH gene and is transmitted as an autosomal dominant trait.

• C1INH is important in regulating the classical complement system and contact cascade .

• Respectively, Insufficient C1INH function leads to dysregulation of each of these pathways.

• However, contact pathway dysregulation is largely responsible for the symptoms of HAE via excess generation of bradykinin

Bradykinin is ultimately formed when high molecular weight kininogen is cleaved by kallikrein. C1INH specifically inhibits bradykinin

formation via inactivation of kallikrein,, factor XIIa, and factor XIIf.

In individuals with C1INH deficiency, production of bradykinin results via unregulated activation of

kallikrein.

Bradykinin binds to the bradykinin-2 receptor, causing vasodilatation, increased vascular permeability, and smooth muscle contraction, all of which lead to symptoms of HAE.

Risk factors

• Age: 30-60 years old

• Other conditions:

1. Asthma

2. Eczema

3. Hives

• Gender: female

• Drinking alcohol

• Taking NSAIDs drugs

• Emotional stress

• Hyperthyroidism

• Menstruation

The signs and symptoms of angioedema

Acute Angioedema:

Large, thick, firm welts

Swelling of the skin

Pain or warmth in the affected areas

Difficulty breathing or swallowing, in severe cases

Hereditary Angioedema:

• Sudden and severe swelling of the face, arms, legs, hands, feet, genitalia, digestive tract and airway

• Abdominal cramping as a result of digestive tract swelling

• Difficulty breathing due to swelling that obstructs your airway

Diagnosis and investigations

The doctor will examine the welts and areas of swelling if they are still present and take a careful medical history to identify possible causes, If the cause isn't apparent from the medical history or symptoms ,the doctor may recommend an allergy skin test.

Prick , patch

(percutaneous) test.  This is the type of

skin test that's usually performed first. Tiny drops of purified allergen extracts are pricked or scratched into the skin's surface. This test is usually performed to identify allergies to pollen, animal dander, foods, insect venom and penicillin.

Intradermal test.

•  Purified allergen extracts are injected into the skin of the arm. Doctors may perform this test if they strongly suspect allergy to an irritant even though your prick test is negative — especially to an irritant to which a future reaction could be life-threatening, such as insect venom or penicillin.

blood test

• Mast cell levels may be elevated if the attack was due to an acute allergic reaction.

• When the doctor suspects hereditary angioedema, he or she may order blood tests to check for depletion of complement factors 2 and 4, that may indicate deficiency of C1-inhibitor

Differential diagnosis

• Food Allergies

• Hymenoptera Stings

• Hypersensitivity Reactions, Delayed

• Latex Allergy

• The most common Differential diagnosis is urticaria, but urticaria, commonly known as hives, occurs in the upper dermis.

• Un like Angioedema which occurs in ?

• the dermis, subcutaneous tissue,  mucosa and submucosal tissues

Treatment

IN ACUTE ANGIOEDEMA:

• Antihistamines e.g. Ranitidine (Zantac)

• 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

IN HEREDITARY ANGIOEDEMA:

• Antihistamines and oral corticosteroid medications — although useful in treating hives and acute angioedema — are often ineffective in treating hereditary angioedema.

• Medications used to treat hereditary angioedema on a long-term basis include certain androgens (male hormones), such as danazol, that help regulate levels of blood proteins.

prevention

• Avoid known triggers. These may include certain foods or medications, or situations such as temperature extremes, that have triggered past allergic attacks.

• Keep a diary. If you suspect foods are causing the problem, keep a food diary. Be aware that some foods may contain ingredients that are listed by less common names on the label.

prognosis

Angioedema that does not affect the breathing may be uncomfortable, but is usually harmless and goes away in a few days.

So, Angioedema had a very good prognosis.

Conclusion

Angioedema is a rapid swelling of the dermis, subcutaneous tissue,  mucosa and submucosal tissues.

Angioedema is classified as either hereditary or acquired.

• It caused by many things such as Animal dander and Food.

• The main sign is Swelling of the skin.

• It can be diagnosed by many tests.

• And it can be easily treated.

• The prevention is mainly by avoid the allergens.

• Finally ,The prognosis is good .

References

• http://www.ncbi.nlm.nih.gov/pubmedhealth/

• http://www.mayoclinic.com

• http://emedicine.medscape.com

Thanks For

Listening