Angioedema or otherwise angioedema (AO) is a pathology characterized by the formation of edema, covering the subcutaneous fat and deep layers of the dermis.

The pathological process may include the mucous layers of the urinary organs, digestive and respiratory systems.

The disease can be inherited, and in some people it is an acquired pathology.

In terms of symptoms, angioedema is similar to Quincke's edema, but it has slightly different development mechanisms, therefore, appropriate treatment should be selected.

Epidemiology

Angioedema is currently being actively studied, it has been established that it occurs in almost 15-25% of people during their lifetime. The disease in half of these cases is recorded in patients with chronic urticaria.

The likelihood of AO (up to 0.1-0.7%) is increased in people receiving treatment with ACE inhibitors.

Hereditary angioedema (HAE) is rarely detected, its occurrence has been established in 2% of people whose parents have had the disease. The development of HAE does not depend on race and gender.

The manifestation of the disease

Angioedema is primarily manifested by a rapidly growing swelling of the surface layer of the epidermis with the involvement of subcutaneous fat, which is clearly visible visually.

With internal spread, edema passes to the digestive organs, the mucous membrane of the bladder, and the respiratory system. In most patients, symptoms disappear within 72 hours after the first signs of AO appear.

Hereditary and acquired forms of angioedema have their own characteristics of development and course.

The clinic of the NAO and the features of its development, which are characterized by

The formation of edematous areas that are not itchy and dense on palpation.

They can be localized in any area of ​​the body, but most often the lips and eyelids, the upper part of the feet and hands, the femoral part of the lower extremities, and the genital area are involved in the pathological process.

When the upper respiratory system is included in the process, swelling begins above the larynx, spreading to the pharynx, oral cavity, surface of the tongue, lips.

Laryngeal edema is one of the most common manifestations of the disease; they are periodically recorded in half of patients with this disease.

The swelling of the larynx is indicated by hoarseness of the voice or its complete absence, noisy breathing, feeling of a lump in the throat. In the absence of timely assistance, increasing suffocation can lead to death.

Less commonly, edema captures the walls of the intestine, indicating this is increasing pain in the abdomen, profuse diarrhea, vomiting, during endoscopy, an area of ​​\u200b\u200bsubmucosal edema is detected that does not have signs of an inflammatory reaction.

In the abdominal variant of AO, the clinical picture is similar to the symptoms of an "acute" abdomen.

In rare cases, edema captures the meninges, which is manifested by symptoms of circulatory disorders in different parts of the brain.

Edema of the urinary organs causes dysuric disorders and urinary retention.

HAE may present with swelling of the muscle layer of the shoulder, neck, back, and swelling of the hip or shoulder joints;

Spontaneity.

Most often, angioedema is caused by trauma, sometimes even minor. Their development can be provoked by psycho-emotional stress, infection, dental and surgical intervention, in women menstruation, low temperatures, vibration.

In half of the cases, HAE occurs due to microtrauma and against the background of surgical intervention:

• The formation of edema mainly with the same localization;
• Slow increase in the main symptoms. Puffiness increases over 36 hours, is resolved in about 3-5 days. Abdominal manifestations usually resolve within a day;
• The absence of a pronounced therapeutic effect from the introduction of antihistamines and glucocorticosteroids;
• relapsing course. Exacerbations in some patients occur almost every week, in others up to several times a year.

The hereditary form of the disease is first recorded before the age of 20 and more often this occurs in adolescence.

The symptoms of the acquired form are identical to the hereditary variant of the disease, but there are some differences:

• For the first time, PAO is recorded in people aged 40 to 50 years;
• Hereditary predisposition is not revealed;
• In acute attacks, the symptoms after the administration of the C1-inhibitor concentrate are slightly reduced compared to HAE;
• A greater therapeutic effect is observed if the patient is prescribed antifibrinolytic agents;
• The development of symptoms of autoimmune and neoplastic diseases is fixed several years after the first episode of acquired angioedema.

Angioedema is not manifested by urticaria with itching, however, in the prodromal period, some patients experience annular erythema.

Accompanying edema with itching, blisters and other symptoms characteristic of atopy indicates an allergic etiology of angioedema.

This variant of AO develops mostly rapidly and is most often mistaken for Quincke's edema.

Symptoms of allergic angioedema are well eliminated by agents with glucocorticosteroids, antihistamines and the introduction of adrenaline.

Edema of an isolated form, provoked by taking ACE inhibitors, appears both at the beginning of treatment with these medications, and after a few months from the start of their use. They are localized in the area of ​​the tongue and lips, the surface of the neck, larynx with pharynx.

The development of intestinal edema is not excluded, which is manifested by abdominal pain, and there may not be external, visible manifestations on the body.

Forecast

• Edema covering the larynx can be fatal if medical assistance is not provided on time;
• If angioedema with urticaria periodically worsens within 6 months, then the risk of their recurrent course in the next 10 years reaches 40%;
• In half of patients with angioedema and chronic urticaria, spontaneous remission is determined;
• Both hereditary and acquired forms of the disease are considered chronic pathologies, but properly selected therapy can prevent acute attacks and significantly improve the patient's life.

Development mechanism

True angioedema occurs due to a deficiency of the C1 inhibitor, a gene responsible for the activity of those protein components of the blood that are involved in the process of its coagulation, control inflammatory reactions, pain, and blood pressure levels.

Insufficient production of C1-inhibitor in most cases occurs due to gene disorders.

Acquired angioedema is the result of accelerated breakdown and consumption of the C1 inhibitor. This happens due to autoimmune failures, malignant neoplasms, and severe infectious processes.

Both hereditary and acquired lack of C1-inhibitor affects the increase in the production of bradykinin and C2-kinin - substances under the influence of which the permeability of the walls of blood vessels increases many times and, accordingly, all conditions for the development of the disease are created.

HAE is divided into 3 types:

1. In the first type, the concentration and functional activity of the inhibitor is reduced by 50 percent or more;
2. In the second type, a normal or elevated level of C1 inhibitor concentration is noted, but its activity is almost halved;
3. In the third type, the mechanism of formation of angioedema is not completely clear. Both the level of the inhibitor itself and its activity remain within the normal range. Scientists associate the occurrence of edema with an increase in the production of bradykinin and with a decrease in its destruction, which occurs when kininase activity decreases under the influence of estrogens.

Angioedema can be of allergic etiology, the development is based on the activation of mast cells, followed by the release of inflammatory mediators, such as leukotrienes, histamine, prostaglandins.

Under their influence, the vessels of the papillary layer of the skin expand, the permeability of the vessels increases, intercellular edema develops, and eosinophils, neutrophils, basophils, and lymphocytes migrate to the inflammatory focus.

This leads to the formation of edema in the subcutaneous fat and in the deep layers of the dermis. Internal swelling may be accompanied by the appearance of itchy blisters on the skin.

The first attack of allergic angioedema is preceded by sensitization of the body to a certain type of allergen - to drugs, food, latex products, insect poisons.

IMPORTANT TO KNOW: How it manifests itself.

Approximately half of patients with a history of angioedema with urticaria in the blood have IgG1, IgG3, IgG4 antibodies to the α-chain of the FceRI receptor. Under their influence, degranulation of basophils and mast cell membranes occurs, which leads to the release of anaphylotoxin C5a.

There is also a special form of acquired angioedema - idiopathic. It is set if the main cause of the pathology cannot be established.

Causes of the disease

With a hereditary form of angioedema, an exacerbation often occurs for no apparent reason.

Puffiness can be triggered by a sharp change in weather conditions, psycho-emotional stress, physical overstrain.

Allergic AO occurs under the influence of one or more allergens.

Indications for consultation with doctors

A patient with suspected angioedema needs to consult doctors of narrow specialties, these are:

• Dentist. If necessary, sanitation of the oral cavity is carried out;
• Gastroenterologist. Pathologies of the gastrointestinal tract are excluded;
• Otorhinolaryngologist. In patients with AO, a high degree of fungal infections and opportunistic microorganisms in the oral cavity is often determined, the development of edema in the larynx is characteristic;
• Surgeon. Consultation of this specialist is necessary for patients with abdominal syndrome;
• Oncologist. The malignant process is excluded;
• Allergist-immunologist;
• Rheumatologist. The consultation of this specialist is especially necessary if the swelling is localized in the area of ​​the articular surfaces.

First aid

Angioedema is considered dangerous for its consequences of diseases, so you need to be able to provide first aid correctly.

With the development of signs of AO, it is necessary:

• Immediately call an ambulance;
• Stop contact with the suspected allergen;
• If swelling has begun after an injection or after an insect bite, then a pressure bandage should be applied to the area above the impact. If the injection is placed in the buttock, then ice or a cold compress is applied to this place. A decrease in temperature leads to vasoconstriction and thus slows down the spread of the allergen throughout the body;
• Release the patient from pressure clothing - unfasten the belt, loosen the tie;
• Provide fresh air by opening windows and doors;
• Calm down the person
• Give to drink (one tablet per 10 kg of body weight) dissolved in warm water.

Indications for hospitalization

Planned hospitalization of patients with AO is carried out during the period of remission, its purpose is to diagnose and identify comorbidities.

The patient is urgently admitted to the hospital:

• When acute symptoms of the disease are detected;
• With severe swelling of the mucous membranes, skin and subcutaneous tissue.

Classification

Angioedema according to the clinical picture is divided into:

• According to the peculiarities of the course, acute (lasting no more than 6 weeks) and chronic (relapses disturb 6 weeks or more);
• According to the cumulative combination with the symptoms of urticaria for the combined and isolated type.

According to the mechanism of its development, it is classified into:

• Pathology with a predominant occurrence under the influence of the activation of the complement system;
• With the participation of other mechanisms;
• idiopathic form.

Hereditary angioedema is divided into three types:

• Type I - the disease is associated with the absolute absence of a c1-inhibitor, characterized by isolated edema;
• Type II - characterized by a relative lack of an inhibitor;
• Type III - there is no lack of C1 inhibitor in the body.

Acquired angioedema:

• Type I - the absolute absence of the C1 inhibitor develops.
• Type II - there is a relative lack of an inhibitor with the formation of autoantibodies to it in the body;

Angioedema not associated with pathological changes in the complement system:

• AO arising from the active release of inflammatory mediators from mast cells. In half of the recorded cases, such angioedema accompanies urticaria, and both diseases are characterized by a common mechanism of occurrence and provoking causes. For their relief, one therapy regimen is used;
• Angioedema resulting from an increase in the activity of kinin mechanisms of blood vessels;
• Episodic - characterized by the development of symptoms of angioedema, urticaria, fever, itching of the skin, weight gain, leukocytosis with eosinophilia in the blood, serum ΙgM increases. Episodic AO are rare and in 80% of cases their outcome is favorable.

Diagnostics

When examining patients, it is necessary to carefully collect anamnesis and complaints.

Patients mainly point to:

• , covering the eyelids, lips, cheeks, scalp and forehead;
• Swelling of the scrotum, limbs, mainly feet, joints, hands;
• Painful sensations in the abdomen with the localization of swelling on the mucous membranes of the digestive tract;
• Swelling inside the larynx, with stenosis, a barking cough appears.

When questioning, it is required to establish whether there were cases and whether there is a genetic predisposition to allergic diseases.

Physical examination of the patient in the acute period establishes:

• Edema with different localization. Edematous areas from healthy areas of the body are sharply limited, the edema itself is dense, pressure on it does not leave pits;
• Cough, choking, hoarse voice, noisy breathing with swelling extending to the larynx;
• Intestinal colic, vomiting and nausea with diarrhea in case of damage to the digestive organs.

Instrumental examination:

• Pulse measurement. With AO, tachycardia is established;
• BP - normal or low;
• HR - bradycardia or tachycardia;
• Heart sounds, muffled;
• Breathing - superficial, slowing down or quickening of respiratory movements.

Examination of the patient should be carried out according to the standard scheme:

• Skin covers. The color is assessed (with AO more often pallor), the degree of puffiness, acrocyanosis;
• Face and head. Inspection is carried out in order to establish traumatic injuries;
• Ears and nose. Pay attention to the release of biological fluids - pus, blood, cerebrospinal fluid;
• Eyes. Assess the condition of the conjunctiva - hyperemia, swelling;
• Neck. There may be swelling of the veins of the neck and the upper half of the body, there is no stiff neck;
• Language. Examine for an increase, evaluate its humidity;
• Rib cage. Exclude injuries;
• Stomach. Assess the size, degree of swelling, tension, exclude the symptoms of an acute abdomen.

IT'S IMPORTANT TO KNOW : ?

Allergic angioedema must be differentiated from HAE.

Table of differential diagnostics.

Treatment tactics

Hereditary angioedema is treated for a long time, therapy includes:

• Relief of acute symptoms;
• Short-term prophylaxis to prevent exacerbations;
• Long-term prevention that reduces the severity of relapses and reduces their frequency.

Non-drug treatment

• Maintaining sufficient airway patency and measures aimed at preventing hypovolemic disorders during acute symptoms of AO;
• Carrying out a tracheostomy or intubation in case of urgent need;
• Monitoring of hemodynamics, prevention of fluid overload in patients with cardiovascular pathologies;

A patient with chronic angioedema should be taught how to behave during an attack:

• With a slight exacerbation, it is important to follow all medical recommendations and carefully record all changes in well-being;
• With swelling of the lips, tongue, signs of suffocation - you need to call an ambulance or contact the nearest medical facility;
• Always carry a medical bracelet with information about your disease and a health "passport" with you.

Medical treatment

The effectiveness of the prescribed therapy is determined by how correctly the form of angioedema is established.

Patients with a hereditary form of angioedema are best helped by:

Patients with type 3 hereditary angioedema do not benefit from C1 inhibitor concentrates and antifibrinolytic drugs.

From an acute attack, such patients are removed using symptomatic therapy, maintaining a constant airway patency and canceling estrogen treatment.

Treatment of the allergic form of AO:

• The introduction of chloropyramine - a first-generation antihistamine. Adult dosage is 20-40 mg per day;
• The use of systemic glucocorticosteroids. They reduce the inflammatory response and reduce the permeability of the vascular walls. - daily dosage of 0.5-1 mg per kilogram of weight. Divide it into two doses, take it orally in the morning. Treatment with glucocorticosteroids is carried out for 5-7 days;
• Plentiful alkaline drink. Promotes the removal of allergens and improves microcirculation. You can use activated carbon or other enterosorbent. Activated charcoal for adults is prescribed 3-6 tablets up to 4 times a day, take it at least an hour before or after a meal;
• With a mild degree of angioedema, first-generation antihistamines are effective. Hifenadine is prescribed 25-50 mg up to 4 times a day, the course of therapy is 10-12 days. On the 4th day, the patient can be transferred to taking antihistamines with prolonged action, these are second-generation drugs, 10 mg orally once a day, 5 mg per day is prescribed;
• The allergic process is stabilized by taking cell membrane stabilizers, this is Ketotifen;
• enzyme preparations. The purpose of their use is to reduce sensitization to food allergens. Adults are prescribed Pancreatin 100 mg before each meal;
• Diuretic drugs are used with an increase in edema. Furosemide is drunk in the morning at a dose of 40 mg, during the day maintenance doses of 20 mg are used;
• Adrenaline is used for severe edema, localization of swelling in the respiratory tract, the gastrointestinal tract. The drug is also effective in hypotension. Adrenaline 0.1% is injected subcutaneously at a dose of 0.01 mg per kilogram of the patient's weight. With inefficiency, the introduction of Adrenaline is repeated after 20-30 minutes;
• To improve microcirculation, infusions of antiplatelet agents and anticoagulants are prescribed. Heparin is introduced;
• If the fungal flora is sown, then antifungal drugs, for example, Fluconazole, are additionally prescribed. The daily dosage of this drug is 50-400 mg. Nystatin is prescribed 4 times a day, 100 thousand units after meals. The course of therapy is 7 days.

If the patient is hospitalized for examination, these medications are not used.

Preventive actions

• Patients with angioedema in the past should be carefully prescribed ATP inhibitor drugs, these are Enalapril, Captopril, Ramipril and angiotensin receptor antagonists, these are Telmisartan, Eprosartan, Valsartan. This rule also applies to patients with a family history of angioedema and individuals receiving immunosuppressive therapy after tissue and organ transplantation;
• Patients with severe food-induced anaphylactic reactions should be warned about the need for continued compliance. Foods that caused an allergic reaction are excluded from consumption. Patients with concomitant pathologies of the gastrointestinal tract, liver, metabolic and neuroendocrine disorders should follow a diet with the exception of foods rich in histamine, histamine liberators, tyramine - seafood, smoked meats, hot spices, tomatoes, canned food, wine.
• Patients with severe anaphylactoid drug reactions should not be treated with drugs with a similar chemical formula. People with a hereditary form of HAE should, if possible, resort to surgical interventions and tooth extractions as rarely as possible. It is necessary to avoid injuries (even minor ones), exposure to low temperatures, psychotraumatic situations, excessive physical activity.
• Women who have undergone AO ​​need to learn how to do without oral contraceptives. They are not prescribed hormone replacement therapy.
• Patients with angioedema should, if possible, avoid the use of plasminogen activators - Altepase, Streptokinase, Actilyse.

Angioedema is a severe pathology, with swelling of the larynx, the outcome can be fatal. With the development of symptoms of the disease, it is important to provide medical assistance in time, so you should not rely on your own strength.

Edema in AO disappears on its own in a maximum of three days. But attacks of the disease can occur up to several times a month, and this disrupts the usual life and negatively affects performance.

Timely diagnosis and compliance with preventive measures can reduce the likelihood of acute attacks to a minimum.