Blood, blood-forming organs-immune system (D50-D90). Histamine-mediated angioedema. Endocrine, nutritional, and metabolic diseases (E00-E90). C1 esterase deficiency – glycoprotein from the serine protease inhibitor group, which has a regulatory effect in the complement system (defense system). Injuries, poisoning and other consequences of external causes (S00-T98). Allergic reactions, unspecified
The following are the most important diseases or complications that may be contributed to by Quincke’s edema (angioedema): Injuries, poisonings, and other sequelae of external causes (S00-T98). Asphyxiation
A comprehensive clinical examination is the basis for selecting further diagnostic steps: General physical examination – including blood pressure, pulse, body weight, height; further: Inspection (viewing). Skin, mucous membranes and eyes [recurrent swelling of: Lips, eyelids, tongue, face, larynx (voice box), extremities, genitals] Auscultation (listening) of the heart. Auscultation of the lungs Palpation (palpation) of … Quincke’s Edema: Examination
Laboratory parameters of 1st order – obligatory laboratory tests. C1-INH concentration (C1 esterase inhibitor = C1-INH, C1 inhibitor)* . C1-INH activity* C4 C1q, CH50, autoantibodies against CH50 – if acquired C1-INH deficiency is suspected. Laboratory parameters 2nd order – depending on the results of the history, physical examination and obligatory laboratory parameters – for differential … Quincke’s Edema: Test and Diagnosis
Therapy goals Decrease of the symptomatology “Long-term prophylaxis should reduce the burden of the disease by preventing or relieving attacks” [HAE guideline: see below]. Therapy recommendations Patients with edema of the head should be hospitalized immediately because of the risk of suffocation. [In severe cases, airway protection is urgently required.] Review of permanent medication due … Quincke’s Edema: Drug Therapy
Medical history (history of illness) represents an important component in the diagnosis of Quincke’s edema (angioedema). Family history Are there any individuals in your family who suffer from recurrent swelling? Social history Is there any evidence of psychosocial stress or strain due to your family situation? Current medical history/systemic history (somatic and psychological complaints). What … Quincke’s Edema: Medical History
Optional medical device diagnostics – depending on the results of the history, physical examination, and obligatory laboratory parameters – for differential diagnostic clarification. Abdominal ultrasonography (ultrasound of abdominal organs) – for recurrent abdominal colic of unclear cause and for patients suffering from recurrent edema (swelling of the skin) that cannot be treated with antihistamines or … Quincke’s Edema: Diagnostic Tests
To prevent Quincke’s edema (angioedema), attention must be paid to reducing individual risk factors. Risk factors Physical – pressure, cold, light, etc. Psychological stress situations Medication ACE inhibitors [>50% of cases with severe angioedema; trigger of acute HAE attacks] Acetylsalicylic acid (ASA). Angiotensin receptor neprilysin antagonists (ARNI) – dual drug combination: sacubitril/valsartan. AT1 antagonists (angiotensin … Quincke’s Edema: Prevention
The following symptoms and complaints may indicate Quincke’s edema (angioedema): Leading symptoms Recurrent edema (water retention/skin swelling) of: Lips Eyelids Tongue Face Larynx (larynx) Extremities Genital Feeling of tension in the affected parts of the skin Pruritus (itching) Painful abdominal discomfort/abdominal cramps → think of: Deficiency or decreased activity of C1 esterase inhibitor (C1-INH). Nausea … Quincke’s Edema: Symptoms, Complaints, Signs
Pathogenesis (development of disease) The common pathway for the development of quincke’s edema (angioedema) is activation of the bradykinin pathway. This peptide is a potent vasodilator that leads to rapidly developing edema in the interstitium: According to the cause, the following forms of Quincke’s edema (angioedema) are distinguished: Histamine-mediated angioedema. Allergic angioedema; occurs in half … Quincke’s Edema: Causes
