Angioneurotic Edema: Symptoms, Treatment, Causes, Diagnosis

2024 Author: Rachel Wainwright | [email protected]. Last modified: 2024-01-15 19:51

Angioedema

The content of the article:

Causes
Kinds
Signs
Diagnostics
Treatment
Prevention

Angioedema (Quincke's edema) is an acute condition characterized by the rapid development of local edema of the mucous membrane, subcutaneous tissue and the skin itself. More often occurs on the face (tongue, cheeks, eyelids, lips) and much less often affects the mucous membranes of the genitourinary organs, gastrointestinal tract, respiratory tract.

Angioedema is a common pathology. It occurs at least once in a lifetime in every fifth person, while in half of the cases it is combined with allergic urticaria.

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Causes

In most cases, angioedema is a manifestation of an immediate-type allergic reaction in response to the ingestion of allergens (stinging insect poisons, medications, food allergens).

Once in the body, allergens trigger the antigen-antibody reaction, which is accompanied by the release of serotonin, histamine and other allergy mediators into the bloodstream. These substances have high biological activity; in particular, they are able to dramatically increase the permeability of the walls of blood vessels located in the submucosal layer and subcutaneous fat. As a result, the liquid part of the blood begins to sweat from the lumen of the vessels, which leads to the development of angioedema, which is both local and widespread.

Angioneurotic edema can also be a manifestation of a pseudo-allergic reaction, which is based on individual hypersensitivity to certain medications or foods. But in this case, there is no immunological stage in the pathological mechanism of edema development.

Angioedema can develop as a complication of therapy with ACE inhibitors or angiotensin II receptor antagonists. This form is usually diagnosed in the elderly. The development of edema in this situation is based on the blockade of angiotensin-converting enzyme by drugs. As a result, the destruction of bradykinin is slowed down and the activity of angiotensin II decreases, which leads to a persistent expansion of blood vessels and an increase in the permeability of their walls.

Allergens that can cause angioedema

Another reason for the development of angioedema is a deficiency of a C1-inhibitor, which regulates the activity of blood proteins responsible for coagulation processes, controlling the activity of inflammatory processes and blood pressure levels, and pain. Deficiency of a C1 inhibitor in the body occurs as a result of insufficient synthesis, which is usually associated with gene disorders. Other reasons for the deficiency of the C1 inhibitor can be its accelerated destruction and consumption. These processes are caused by some infectious diseases, malignant neoplasms, and autoimmune pathologies. Acquired or hereditary C1-inhibitor deficiency leads to increased production of C2-kinin and bradykinin, substances that increase the permeability of the walls of blood vessels and contribute to the development of angioedema.

Kinds

Depending on the duration of the pathological process, acute and chronic angioedema is distinguished. The transition of the state to a chronic form is evidenced by its duration over 1.5 months.

Angioedema can be combined with urticaria or be isolated.

Based on the characteristics of the developmental mechanism, hereditary and acquired types of angioedema are distinguished. Acquired, in turn, are subdivided as follows:

allergic;
pseudo-allergic;
associated with the use of ACE inhibitors;
associated with autoimmune processes and infectious diseases.

The idiopathic form is also distinguished. They talk about it when it is not possible to establish the cause of the pathological permeability of the vascular wall.

Signs

In most cases, angioedema develops acutely within 3-4 minutes. An increase in angioedema over a period of 2–5 hours is much less common.

Usually it is localized in the area of the lips, cheeks, eyelids, oral cavity, and in men - even in the scrotum. The clinical picture is largely determined by the site of localization. So, with edema of the submucous layer of the gastrointestinal tract, the patient experiences the following symptoms:

stomach ache;
nausea;
vomiting;
stool disorders.

With angioedema of the larynx, the patient develops characteristic stridor breathing, speech disorders and hoarseness are noted.

Angioneurotic edema of other localizations is much less common:

pleura (characterized by general weakness, shortness of breath, pain in the chest);
the lower part of the urinary system (leads to painful urination, acute urinary retention);
brain (signs of transient cerebrovascular accident are diagnosed);
joints;
muscles.

In 50% of cases, allergic and pseudo-allergic angioedema is accompanied by the development of urticaria, anaphylactic shock.

Distinctive features of hereditary and acquired angioedema:

Signs	Hereditary angioedema	Allergic angioedema
Onset of the disease	In children	More often in adults
Heredity	Yes	No
Allergy anamnesis	No, rarely	Part
Trauma connection	Yes	No
Relationship with allergen exposure	No	Yes
Time of onset of edema	Forms within hours	Occurs over several minutes to 1 hour
Localization of edema	Most often the upper respiratory tract and gastrointestinal tract	Various; in 25% of cases - laryngeal edema
Hives	No	there is
Itching	No	there is
The effect of antihistamines and corticosteroids	No	there is
Blood eosinophilia	No	Part
IgE	Norm	Promoted

The hereditary form is clinically manifested before the age of 20. In this case, the edema grows slowly and undergoes a reverse development within a week. Most often it is localized in the submucous layer of either the larynx or the organs of the digestive system. Hereditary angioedema is prone to frequent relapses, which occur several times a year under the influence of a variety of provoking factors.

Diagnostics

With the localization of angioedema in the face or other open areas of the body, diagnosis is usually not difficult, but in some cases differential diagnosis is required with edema caused by other reasons:

dermatomyositis;
kidney disease;
syndrome of compression of the superior vena cava;
hypothyroidism.

It is quite difficult to make a correct diagnosis in angioedema of the brain and digestive tract organs, since in these cases the symptoms of a transient cerebrovascular accident or "acute abdomen" respectively come to the fore.

Laboratory diagnostics for angioedema is rather auxiliary. In blood tests with an allergic type, an increase in the level of immunoglobulins of class E, eosinophilia are noted. Non-allergic edema is characterized by a decrease in the activity and level of a C1-inhibitor in the blood serum, and the detection of laboratory signs of lymphoproliferative or autoimmune diseases.

Treatment

The greatest danger is angioedema of the larynx, which can cause asphyxia and death of the patient. In this case, it is necessary to urgently restore the patency of the airways (using an air duct, tracheal intubation, conicotomy).

With an allergic form of pathology, the introduction of antihistamines, glucocorticoid hormones, the appointment of enterosorbents, and infusion therapy are indicated.

In the acute period of hereditary angioedema, administration of a C1-inhibitor is indicated. In the absence of this drug, transfusion of native fresh frozen plasma is performed. Androgens and antifibrinolytic agents are also prescribed. With severe edema, especially localized in the neck, diuretics and glucocorticoid hormones are injected intravenously.

During the period of remission of hereditary angioedema for the prevention of relapse, a maintenance course of androgens and antifibrinolytic agents is continued.

First aid for Quincke's edema before the arrival of an ambulance:

Sequencing	Assistance measures
Step 1	Help the patient to sit in a comfortable position
Step 2	Limit the action of a potential allergen, if known. For example, when a wasp bites, it is advised to carefully pull out the sting of an insect from the skin.
Step 3	Give an antihistamine, such as diphenhydramine or diazolin. If possible, it is better to give an injection, because with the development of edema of the gastrointestinal tract, absorption of the drug may be difficult
Step 4	Be sure to give the patient an alkaline drink, for example, narzan, Borjomi or 1 g of baking soda diluted in a liter of water. This will help flush the allergen out of the body. Activated carbon and Enterosgel have the same effect.
Step 5	Place ice or a cooling compress on the swollen area to reduce swelling and itching
Step 6	Facilitate the patient's breathing by creating unhindered access to fresh air, freeing the upper body from the dragging parts of the clothing

Prevention

To prevent recurrent allergic angioedema, the patient should adhere to a hypoallergenic diet, do not take any medications without a doctor's prescription.

To prevent recurrence of hereditary angioedema, patients must avoid stressful situations, viral infections, traumatic injuries. They should not take estrogen-containing medications or ACE inhibitors.

Patients suffering from hereditary angioedema, before conducting planned surgical interventions or dental procedures, must necessarily carry out preventive treatment, including the appointment of tranexamic acid, infusion of fresh frozen native plasma.

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Elena Minkina Doctor anesthesiologist-resuscitator About the author

Education: graduated from the Tashkent State Medical Institute, specializing in general medicine in 1991. Repeatedly passed refresher courses.

Work experience: anesthesiologist-resuscitator of the city maternity complex, resuscitator of the hemodialysis department.

The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!