Angiootek – the pathological state which is followed by accumulation of liquid in tissues of skin and hypodermic fatty cellulose because of increase in permeability of walls of vessels of the microcirculator course. It is shown by the hypostasis of skin of various localization (a face, a neck, extremities) which is quite often combined with urticaria and a skin itch. Diagnostics is made by fizikalny survey, laboratory blood tests, studying of the hereditary and allergologichesky anamnesis of the patient. Therapeutic tactics depends on the reasons which caused development of a syndrome can include purpose of antihistamines, androgens, inhibitors of a fibrinoliz and diuretics.
Angiootek was for the first time described in 1882 by the German scientist Heinrich Quincke. The researcher considered it an independent disease – angioneurotic hypostasis. Now it is established that about a half of cases of pathology arises owing to allergic processes of reaginovy type whereas the others are the independent acquired or hereditary states. Carry a number of diseases which cornerstone violations of circulation of liquid between blood system and fabrics are to the concept "isolated ". Can be diagnosed by Angiooteki at any age, at women come to light about 1,5-2 times more often, than at men. Genetically caused options of a pathological state are transferred on autosomno-prepotent type.
Reasons of an angiootek
There is a huge number of the external and internal factors promoting development of angioneurotic hypostasis. Changes in system of a complement and violation of some other physiological processes (coagulations of blood, fibrinolytic and kininovy reactions) are an immediate cause of pathology. In most cases anomaly is caused by deficiency or insufficient activity of C1 inhibitor – the protease of the blood which is slowing down and stopping a number of biochemical reactions in blood and fabrics. This phenomenon arises under the influence of the following factors:
- Genetic features. A considerable part of episodes of a congenital angiootek is caused by a mutation of a gene of SEPRING1 located on the 11th chromosome. It codes the proteinaceous sequence of C1 inhibitor therefore at change of this gene deficiency of protease or loss of its function is noted.
- Limfoproliferativny pathologies. Some states with the accelerated reproduction of lymphocytes or their cages predecessors (for example, lymphoma) can be followed by angioneurotic hypostases. The reason of it is covered in increase in speed of destruction of C1 inhibitor.
- Autoimmune reactions. The antibodies to C1-esteraze connecting this enzyme and promoting its destruction are under certain conditions formed. Process can arise as separately, and at system allergic reactions or other violations.
Rare option of pathology is , C1 inhibitor developing at optimum level – generally so-called estrogenzavisimy hypostases carry to it. Assume that they are caused by genetic factors and are transferred on the mechanism linked to the H-chromosome, the course of a disease is aggravated at estrogen administration of drugs. Also there is a kind of a disease provoked by use of inhibitors angiotensin - the turning enzyme, the numerous antigipertenzivny means which are a part.
The deficiency of C1 inhibitor arising for any of several reasons leads to activation of system of a complement, increases concentration of a kallikrein and bradikinin. The last stimulate formation of the vazoaktivny peptide connections influencing vascular walls and smooth muscles. Expansion prekapillyarny , then – an exit of elements of plasma of a cage from the blood course in intercellular space results at first. Thus, it is formed local , shown a characteristic clinical picture. Besides, reactions of a bradikininovy link can cause a spasm of gladkomyshechny cages of digestive and respiratory systems, breaking their motility.
Taking into account features of a clinical current all cases of an angiootek divide into two big groups – isolated and combined. The first are shown only by hypostasis of hypodermic fatty cellulose and skin, the second can be followed by a small tortoiseshell, a spasm of airways and other symptoms. Division is rather conditional, poorly reflects causes of illness. On an etiologichesky sign allocate the hereditary and acquired forms of angioneurotic hypostasis. Congenital versions make about 2-5% of total of cases of a disease, include the following types:
- Type 1. It is caused by almost total absence of C1 inhibitor, arising at SEPRING1 gene mutation. It is characterized by quite heavy current – a generalizovannost and expressiveness of hypostases, their emergence not only on skin, but also on mucous airways or a digestive tract. Is registered in 85% of cases of a hereditary angiootek.
- Type 2. Develops at a relative lack of inhibitor owing to its slow education or the lowered activity because of the wrong structure of enzyme. The clinic is less heavy, hypostases affect mainly tissues of extremities, sometimes persons. Such option of a disease is diagnosed for 12-14% of patients with hereditary forms of angioneurotic hypostasis.
- Type 3. Meets extremely seldom, usually – against the background of lack of deficiency C1-esterazy. As a rule, it is presented by estrogenzavisimy hypostases – exacerbations of pathology at pregnancy, reception of the combined oral contraceptives, replacement therapy in the period of a climax.
All options of a hereditary angiootek are isolated, are not followed by a small tortoiseshell or other violations. The acquired versions have other classification including only two main types of a disease:
- Type 1. Comes to light against the background of limfoproliferativny states – lymphoma, at some infectious defeats. Increase in consumption C1 inhibitor and its subsequent shortcoming becomes the reason.
- Type 2. Arises because of synthesis autoantitet to inhibitor that sharply reduces its concentration in blood. Such phenomenon takes place at some autoimmune and allergic states, immunodeficiencies and other pathologies.
Symptoms of an angiootek
The main symptom of pathology is emergence of painless hypostasis of skin of various size. Patients note feeling of a raspiraniye and tension, other subjective complaints are absent. Unlike inflammatory hypostases the struck area is characterized by more pale, than surrounding sites of skin, color and lack of local temperature increase of fabrics. Most often edematous manifestations are found on the top and lower extremities, the person (lips, cheeks, eyelids, auricles), a neck, in a zone of genitals. The skin itch is uncharacteristic, but can be defined at a combination of an angiootek to a small tortoiseshell.
At some patients hypostasis comes to light in mucous membranes, sometimes pathological process affects also a submucous plate. Most often bodies of an oral cavity (language, a soft palate), airways, digestive tract are surprised. At development of an angiootek of bodies of respiratory system there is a feeling of shortage of air, an osiplost or total loss of a voice, the barking cough. Involvement of a GIT is shown by the expressed abdominal syndrome – pains, nausea, vomiting. Tension of muscles of a belly wall creating a false picture of peritonitis or sharp intestinal impassability is noted.
Extremely rare symptoms of a disease are signs of a pleural exudate (cough, breast pain, the complicated breath). Other rare options of pathology local hypostasis of a brain (consciousness oppression, a hemiparesis is registered), is among a bladder (is followed by a sharp delay of urine), damage of muscles and joints. The isolated forms of a disease develop slowly for 12-48 hours. After that in the absence of complications there is slow permission of puffiness within 5-8 days. Some combined options of angioneurotic hypostasis (especially allergic genesis) can progress much quicker – within several minutes or hours.
The probability of complications at an angiooteka depends on localization of pathological process. Most often (approximately in a half from all complicated cases) patients have breath difficulties caused by narrowing of a gleam of a throat or bronchial tubes. In the absence of medical care violation can become the reason of a lethal outcome. Abdominal forms of pathology which can cause frustration of a vermicular movement with development of impassability and peritonitis are rather dangerous. Quite often hypostases of bodies of a GIT become the reason of unnecessary surgical intervention because of wrong diagnostics. Damage of a brain is capable to lead to a coma and a number of neurologic consequences (an incoordination, the speech, perception). The sharp delay of urine at the edematous phenomena in a bladder is the reason of a reflux of liquid, a gidronefroz and a renal failure.
In most cases the diagnosis angioneurotic hypostasis is exposed by the immunologist. Less often experts of other directions – dermatologists, pediatricians, gastroenterologists, therapists face this pathology. Definition of a disease is often complicated because of its various etiology and very wide range of clinical manifestations. The main attention is paid to anamnestichesky information and results of specific laboratory researches. Diagnostics of an angiootek includes the following techniques:
- Poll and survey. At external examination specify prevalence and localization of the edematous site, confirm lack of morbidity. By method of inquiry find out what preceded development of pathological manifestations (a stress, the use of any products, drug intake) whether similar reactions at relatives took place.
- Laboratory analyses. A specific method of diagnostics of an angiootek is determination of the C1 inhibitor level of plasma of blood – its absence or decrease in quantity testifies in favor of existence of a disease. Definition of a caption of antibodies to C1 inhibitor is possible – this technique allows to find out whether the acquired autoimmune character has a disease.
- Additional researches. At defeat of respiratory system make a bronkhoskopiya, a X-ray analysis of bodies of a thorax. Usually hypostasis of tissues of throat, a bronchospasm comes to light, the pleural exudate occasionally is found. Ultrasonography of abdominal organs allows to differentiate abdominal forms of an angiootek from peritonitis and other pathologies of bodies of a GIT.
Besides above-mentioned methods at diagnostics of this state consider a huge number of various factors. For example, age of the patient: hereditary versions are found in persons more often 20 years are younger, the acquired forms – at people are more senior than 40 years with the burdened anamnesis. Take into account existence or lack of the accompanying symptoms – small tortoiseshells, respiratory frustration. Differential diagnostics is carried out with hypostases of other genesis – as a result of renal pathology, a sting of poisonous insects, local allergic and inflammatory reactions.
Treatment of an angiootek
Therapeutic actions at angioneurotic hypostasis divide into two groups – methods for knocking over of a bad attack and the equipment for prevention of its subsequent development. In both cases use similar medicinal substances – depending on the purposes of their appointment only the scheme of reception and a dosage changes. Most often in modern immunology apply the following medicines to treatment of an angiootek:
- Androgens. Some analogs of male sex hormones (, methyltestosterone) are capable to strengthen synthesis C1-esterazy in cells of a liver. They reduce expressiveness of symptoms of pathology and reduce probability of an attack of disease in the future.
- Inhibitors of a fibrinoliz. The medicines interfering fibrinolytic processes also slow down reactions of a kallikreinovy way. Thanks to it the speed of diffusion of plasma in fabric decreases, the probability of emergence of an angiootek decreases. Use of medicines of this group (e-aminokapron or traneksamovy acids) is carried out under control of a condition of the curtailing system of blood.
- Freshly frozen plasma. Transfusion of the donor plasma containing C1 inhibitor is an effective method of knocking over of sharp hypostases, especially hereditary character.
In the presence autoantitet against components of a complement their removal from a blood-groove by means of a plasma exchange is shown. It is the temporary measure capable it is considerable to weaken expressiveness of edematous manifestations. At threat of life of the patient (for example, because of an obturation of airways) adrenaline introduction is recommended, and at its inefficiency – koniko-or tracheotomy. If existence of other disease (allergic, autoimmune or other character) served as the reason of an angiootek – the scheme of its treatment according to indications is developed. There are also perspective medicines of inhibitor which are used in some countries for therapy of this state.
Forecast and prevention
The forecast of an angiootek is considered uncertain before clarification of its etiology at the specific patient. At the hereditary nature of pathology there is always a risk of development of lethal hypostasis of a throat therefore it is desirable for patients to have at itself a card with the indication of the diagnosis. At the correct preventive treatment attacks arise seldom, do not pose a threat for the patient's life. The forecast of the acquired forms depends on the nature of the main disease. Preventive actions include timely treatment of allergic and autoimmune states.