Angioneurotical edema code per micro-10
Angioedema - sharply developing, rapidly passing edema of the skin and subcutaneous tissue or mucous membranes.
Etiology. Angioedema, a polyethyological disease, can be caused by both immune and non-immune factors.
• Allergic - manifestation of type I allergic reaction; develops as a manifestation of sensitization to drugs (more often antibiotics), food products and additives, poison stinging insects (bees, wasps and hornets).
• Pseudoallergic - as a consequence of direct non-immune histamine-releasing effect of some drugs (salicylates and other NSAIDs, ACE inhibitors, dextrans, etc.), food or supplements (see Food Allergy).
• Complement-dependent - can be inherited or acquired (asin patients with lymphoproliferative malignancies, which is associated with an acceleration of the metabolism of the inhibitor of esterase C1 of the complement component by a factor of 2-3).
• Idiopathic - The etiology is unclear.
Risk factors • Atopic predisposition • ReceptionACE inhibitors (in 0.2% of cases) • Presumably, taking omeprazole (a proton pump inhibitor) and sertraline (selective serotonin reuptake inhibitor).
Genetic aspects. Hereditary angioedema (* 106100,insufficiency of the esterase inhibitor of the complement component C1, 11p11.2-q13, deletion of the C1NH gene in type 1, point mutation in type 2, ) is characterized by recurrent episodes of edema of the skin, upper respiratory tract and gastrointestinal tract (up to intestinal obstruction).
• C1q-inhibitor - highly glycosylated proteinserum synthesized in the liver and inhibiting the proteolytic activity of the C1 and C1s subcomponents, thereby preventing the activation of C4 and C2 complement components.
• Insufficiency of the C1q-inhibitor leads touncontrolled activation of early complement components and the formation of a kinin-like factor that causes an increase in vascular permeability and leads to the development of angioedema.
• The most common (in 80-85% of cases) is the causehereditary angioedema, a quantitative deficiency of the C1q inhibitor (type 1); in other cases, with a normal amount of inhibitor, the cause of hereditary angioedema is its functional inferiority to the C1 inactivator due to a point mutation in the reactive center of the molecule (type 2).
Pathogenesis similar to the pathogenesis of urticaria and associated withdilation and increase in vascular permeability (mainly venules) of deep layers of dermis and submucosal layer caused by mediators of allergy (histamine, Pg, leukotrienes), as well as complement components (primarily C3a and C5a) and kinins.
Pathomorphology. Edema, vasculitis and / or perivasculitis involving only subcutaneous tissue.
Clinical picture • Local edema of the skin, subcutaneous tissue and / ormucous membranes in combination with or without urticaria • Often - a component of the generalized anaphylactic reaction (anaphylactic shock) • May develop in any part of the body; usual localization - a person (usually lips, eyelids), limbs, external genitalia. The greatest danger to life is the laryngeal edema and the asphyxia caused by it • Rapid onset and rapid spontaneous resolution • The feeling of numbness is characteristic, the itching, expressed much weaker in comparison with hives.
Laboratory research • With allergic form, the IgEby means of a radioallergosorbent test (especially for penicillin allergy and food allergens). • Determination of C1q-inhibitor levels, as well as of C2 and C4 complement components in hereditary angionevrotic edema. • Reduction of C1q-inhibitor level below 100 mg / ml for type 1 • • Decrease in C4 for both types 1 and 2 •• C2 decreased with exacerbation at types 1 and 2 • • In patients with acquired forms of C1-inactivator deficiency, C1 (in contrast to congenital forms) was also decreased.
Differential diagnostics • Diffuse subcutaneous infiltrative process •Contact dermatitis • Lymphostasis (lymphatic edema) • Thrombophlebitis • Local edema with somatic pathology • Granulomatous cheilitis • Erysipelas with pronounced edema • Cellulitis.
Diet. Exclusion of known food allergens (egg whites, milk, wheat, fish, nuts, tomatoes, chocolate, bananas, citrus fruits) until the transition to the basic hypoallergenic diet.
Tactics of reference • Protection from the effects of known provoking factors • Cold compress to reduce the intensity of itching • Intubation with upper airway obstruction.
Drug therapy. Drugs of choice - see Hives. Special care! Angioneurotic edema of the larynx and the asphyxia caused by it are potentially life threatening and require active therapy. • Epinephrine 0.3 ml 0.1% of the r-ra sc, and locally as an aerosol • HK-dexamethasone 4-8 mg IM or IV, or 30-60 mg of prednisolone; with hereditary angioedema edema the effectiveness of HA is doubtful • Antihistamines - dimethindene IV, diphenhydramine, clemastin in / m 1-2 ml.
Complications. Asphyxia due to airway obstruction.
Current and forecast. Most patients with angioedemaedema, the general condition suffers insignificantly, with the exception of cases of airway obstruction. In chronic forms, the course and prognosis depend on the etiology and pathogenesis.
Concomitant pathology • Anaphylaxis • Hives.
Prevention • Avoid contact with a causal factor •ACE inhibitors are contraindicated • Before the procedures that could provoke the development of hereditary angioedema (dental procedures, intubation, endoscopy, etc.), danazol is recommended at 200 mg 3 r / day 3 days before the procedure, and immediately before it - the introduction of 2 standard packages fresh frozen plasma • For long-term prophylaxis: •• Danazol 200-600 mg daily for 1 month, then for 5 days every 5 days. Danazol is ineffective in other forms of C1-esterase inhibitor deficiency; contraindicated in children, pregnant, nursing mothers, with porphyria; can cause headache, weight gain, hematuria •• Aminocaproic acid 1 g 3 r / day.
Synonyms • Quincke's disease • Quincke's edema.
ICD-10 • T78.3 Angioedema • D84.1 Defect in the system