Quincke swelled - it is sharply arising disease which is characterized by emergence of accurately limited angiootek of skin, hypodermic cellulose, and also a mucous membrane of various bodies and systems of an organism. The major causal factors – a true and false allergy, infectious and autoimmune diseases. Angiootek sharply arises and passes within 2-3 days. Medical actions at Quincke's hypostasis include knocking over of complications (restoration of passability of airways), performing infusional therapy (including C1 inhibitor and aminocaproic acid at hereditary hypostases), introduction of glucocorticoids, antihistaminic medicines.
Quincke () – sharply developing local puffiness of skin, hypodermic cellulose, mucous membranes of the allergic or pseudo-allergic nature most often arising on a face swelled (on lips, centuries, a cheek, language), is more rare – on mucous membranes (airways, digestive tract, urinogenital bodies). At development of hypostasis of Quincke in the field of language and a throat passability of airways can be broken and arise threat of asphyxia. The hereditary form, at 30% - acquired in other cases is diagnosed for 25% of patients it is not possible to reveal a causal factor. On statistical data, during life Quincke swelled occurs approximately at 20% of the population, and in 50% of cases is combined with a small tortoiseshell.
Reasons of hypostasis of Quincke
Acquired swelled Quincke quite often develops in response to penetration into an allergen organism – medicamentous means, foodstuff, and also at stings and uzhaleniye of insects. The acute allergic reaction arising at the same time with emission of mediators of an inflammation increases permeability of the vessels located in hypodermic and fatty cellulose and a submucous layer and leads to emergence of local or widespread hypostasis of fabrics on a face and in other places of an organism. Quincke swelled can develop also at a pseudo-allergy when hypersensitivity to some medicines, foodstuff and additives to food develops in the absence of an immunological stage.
One more of the causal factors promoting developing of hypostasis – reception of such medicines as APF inhibitors (captopril, enalaprit), and also antagonists of receptors of angiotensin II (a valsartan, an eprosartan). In this case angiootek are observed generally at elderly people. The mechanism of developing of hypostasis at the use of these medicines is caused by blockade of angiotenzinprevrashchayushchy enzyme therefore vasoconstrictive effect of hormone of angiotensin II decreases and destruction of a vazodilatator of a bradikinin is slowed down.
Quincke swelled can develop also at the congenital (hereditary) or acquired deficiency of C1 inhibitor regulating activity of system of a complement, fibrillation and a fibrinoliz, kallikrein-kininovy system. At the same time deficiency of C1 inhibitor arises both at its insufficient education, and at the increased use and insufficient activity of this component. At hereditary hypostasis as a result of gene mutations the structure and the C1 inhibitor function is broken, there is an excess activation of a complement and Hageman's factor, and as a result – the increased formation of a bradikinin and C2-kinina which increase vascular permeability and is led to formation of angiootek. Acquired Quincke caused by deficiency of C1 inhibitor swelled, develops at its accelerated expenditure or destruction (development autoantitet) at malignant new growths of lymphatic system, autoimmune processes, some infections.
Sometimes the option of hereditary hypostasis of Quincke meets the normal C1 inhibitor level, for example, at a family mutation of a gene of a factor of Hageman, and also at women when the strengthened production of a bradikinin and his slowed-down destruction it is caused by braking of activity of APF estrogen. Quite often various causal factors are combined among themselves.
Classification of hypostasis of Quincke
On clinical manifestations distinguish the sharp course of hypostasis of Quincke continuing less than 1,5 months and a chronic current when pathological process continues 1,5-3 months and longer. Allocate the angiootek isolated and combined with a small tortoiseshell.
Depending on the mechanism of development of hypostasis distinguish the diseases caused by violation of regulation of system of a complement: hereditary (there is an absolute or relative deficiency of C1 inhibitor, and also its normal concentration), acquired (with deficiency of inhibitor), and also , developing at the use of APF inhibitors, owing to an allergy or a pseudo-allergy, against the background of autoimmune and infectious diseases. Allocate and idiopathic swelled Quincke when it is not possible to establish the concrete reason of development of an angiootek.
Symptoms of hypostasis of Quincke
Quincke swelled develops, as a rule, sharply within 2-5 minutes, less often can gradually be formed with increase of symptomatology for several hours. Typical places of localization – parts of the body where there is a friable cellulose: in the field of a century, cheeks, lips, on a mucous membrane of an oral cavity, in language, and also on a scrotum at men. If hypostasis develops in areas of a throat, there is a voice osiplost, the speech is violated, there is a whistling stridorozny breath. Development in a submucous layer of a digestive tract leads to a picture of sharp intestinal impassability – to emergence of severe pains in a stomach, nausea, vomiting, frustration of a chair. Meets much less often Quincke with damage of a mucous membrane of a bladder and urethra (the delay of urine, pain at an urination is noted), pleurae (thorax pains, short wind, the general weakness), a brain (symptoms of passing violation of brain blood circulation), muscles and joints swelled.
Quincke swelled at an allergic and pseudo-allergic etiology in half of cases is followed by a small tortoiseshell with a skin itch, blisters, and also can be combined with reactions from other bodies (a nose cavity, bronchopulmonary system, a GIT), to be complicated by development of anaphylactic shock.
The hereditary hypostasis connected with violation of work of system of a complement, as a rule, arises aged up to 20 years, is shown by slow development of symptoms of a disease both their increase within a day and gradual return development within 3-5 days, frequent damage of a mucous membrane of internals (an abdominal syndrome, throat hypostasis). Quincke owing to inherited disorders swelled tends to a retsidivirovaniye, repeating from several times in a year to 3-4 times a week under the influence of the most various provocative factors – mechanical damage of integuments (mucous membrane), a catarrhal disease, a stress, alcohol intake, estrogen, APF inhibitors etc.
Diagnosis of hypostasis of Quincke
The characteristic clinical picture typical for Quincke's hypostasis with localization on a face and other open parts of the body, allows to establish the correct diagnosis quickly. More difficult a situation at emergence of a picture of "a sharp stomach" or the tranzitorny ischemic attack when it is necessary to differentiate observed symptoms with a number of diseases of internals and nervous system. It is even more difficult to differentiate hereditary and acquired , to reveal the concrete causal factor which caused its development.
Careful collecting anamnestichesky data allows to define hereditary predisposition in respect of allergic diseases, and also presence at relatives of the patient of cases of hypostasis Quincke without identification at them any allergoz. It is worth asking also on cases of death of relatives from suffocation or frequent to surgeons concerning attacks of repeated severe pains in a stomach without performing any surgeries by it. It is also necessary to find out whether the patient had any autoimmune or oncological disease, whether it accepts APF inhibitors, blockers of receptors of angiotensin II, estrogen.
The analysis of complaints and data of survey quite often allows to differentiate approximately hereditary and acquired Quincke swelled. So, slowly accruing are characteristic of hereditary angiootek and it is long the remaining hypostases often affecting mucous a throat and a digestive tract. The symptomatology quite often appears after an insignificant trauma at young people in the absence of any communication with allergens at what antihistaminic medicines and glucocorticoids are inefficient. At the same time there are no other displays of an allergy (a small tortoiseshell, bronchial asthma) that is characteristic of hypostases of an allergic etiology.
Laboratory diagnostics at angiooteka of not allergic nature allows to reveal decrease in level and activity of C1 inhibitor, autoimmune pathology and limfoproliferativny diseases. At Quincke's hypostasis connected with an allergy the blood eozinofiliya, increase in level of the general IgE, positive skin tests comes to light.
In the presence of stridorozny breath at hypostasis of a throat carrying out a laringoskopiya can be required, at an abdominal syndrome – attentive survey of the surgeon and carrying out necessary tool researches, including endoscopic (a laparoscopy, a kolonoskopiya).
Differential diagnosis of hypostasis of Quincke is carried out with other hypostases caused by a hypothyroidism, a syndrome of a sdavleniye of the top hollow vein, pathology of a liver, kidneys, dermatomiozity.
Treatment of hypostasis of Quincke
First of all at Quincke's hypostasis of any etiology it is necessary to eliminate threat for life. It is for this purpose important to restore passability of airways, including by an intubation of a trachea or a konikotomiya. At an allergic angiootek glucocorticoids, antihistaminic medicines are entered, the contact with potential allergen is eliminated, infusional therapy, an enterosorbtion is carried out.
At Quincke's hypostasis of hereditary genesis during the sharp period introduction of C1 inhibitor (in the presence), freshly frozen native plasma, anti-fibrinolytic medicines (aminocaproic or traneksamovy acid), androgens (a danazol, a stanozol or methyltestosterone) is recommended, and at an angiooteka in a face and a neck – glucocorticoids, furosemide. After improvement of a state and achievement of remission treatment by androgens or antifibrinolitika is continued. Application of androgens is contraindicated at children's age, at women during pregnancy and feeding by a breast, and also at men with malignant tumors of a prostate gland. In these cases use intake solution aminokapron (or traneksamovy) acids in individually picked up doses.
The patient with hereditary hypostasis Quincke before holding dental procedures or surgeries as short-term prevention recommends reception of traneksamovy acid two days prior to operation or androgens (in the absence of contraindications) in six days prior to the surgical procedure. Just before invasive intervention it is recommended to carry out infusion of native plasma or aminocaproic acid.
Forecast and prevention of hypostasis of Quincke
The outcome of hypostasis of Quincke depends on weight of manifestations and timeliness of holding medical actions. So throat hypostasis in the absence of emergency aid comes to an end with death. The recurrent urticaria which is combined with Quincke's hypostasis and proceeding within six months and more at 40% of patients is observed further on an extent of 10 years, and at 50% there can occur long remission even without the supporting treatment. Hereditary angiootek periodically recur throughout all life. Correctly picked up supporting treatment allows to avoid complications and considerably improves quality of life of patients with Quincke's hypostasis.
At allergic genesis of a disease it is important to observe hypoallergenic food, to refuse reception of potentially dangerous medicines. At a hereditary angiootek it is necessary to avoid damages, viral infections, stressful situations, reception of APF inhibitors, estrogensoderzhashchy means.