From the Guidelines
Quincke's edema, also known as angioedema, should be treated with C1-INH, icatibant, or ecallantide as early as possible to minimize duration and severity, especially in cases of upper airway edema which should be treated as a medical emergency. The treatment of Quincke's edema depends on the severity and location of the swelling. For hereditary angioedema, the primary mediator of swelling is bradykinin, and the treatment should focus on inhibiting this mediator 1.
Treatment Options
- C1-INH, icatibant, or ecallantide are the recommended treatments for acute attacks of hereditary angioedema, and they should be administered as early as possible to minimize the duration and severity of the attack 1.
- In cases of upper airway edema, early intubation or tracheotomy may be necessary to maintain airway patency, and patients should be closely monitored for signs and symptoms of impending airway closure 1.
- Symptomatic treatment may also be necessary, including pain medication, antiemetics, and aggressive hydration for abdominal attacks, and close monitoring for signs of airway compromise in oropharyngeal and laryngeal attacks 1.
Important Considerations
- Patients with hereditary angioedema should carry an acute attack treatment with them and be trained on self-administration, with the exception of ecallantide 1.
- The management of individual attacks can also involve symptomatic treatment based on the region of body swelling, and the observation period should be individualized 1.
- Narcotic addiction is a risk for patients with hereditary angioedema who experience frequent attacks, and out-of-hospital use of potent narcotics should be avoided 1.
From the Research
Definition and Causes of Quincke's Edema
- Quincke's edema, also known as angioedema, is a self-limiting edema of the subcutaneous or submucosal tissues due to localized increase of microvascular permeability whose mediator may be histamine or bradykinin 2.
- It can be caused by various factors, including allergic reactions, exposure to allergens and drugs (mainly ACE inhibitors and non-steroidal anti-inflammatory drugs), and family history of similar symptoms 2, 3.
- Quincke's edema can also be referred to as angioneurotic edema involving the uvula, and can be secondary to food allergy 4.
Diagnosis and Treatment
- The diagnosis of Quincke's edema is primarily clinical, with laboratory tests used to confirm the diagnosis 2.
- Treatment options for Quincke's edema include standard therapies such as epinephrine, glucocorticoids, and antihistamines for allergic histaminergic angioedema 2.
- For non-histaminergic angioedema, therapeutic options include C1-inhibitor concentrates, icatibant, and ecallantide, with fresh frozen plasma considered if these drugs are not available 2, 3.
- In cases of ACE inhibitor-induced angioedema, off-label use of icatibant has shown promise, with rapid symptom resolution and avoidance of intubation and tracheotomy 3, 5.
Management of Serious or Life-Threatening Cases
- In serious or life-threatening cases of Quincke's edema, such as those involving the larynx, immediate intervention is crucial to prevent asphyxiation 6.
- Replacement therapy with C1-inhibitor concentrate (C1-INH) has proven effective in cases of hereditary angioedema (HAE) 6.
- The challenge is to realize whether an airway obstruction is due to HAE or not, and careful observation, the right medication, and appropriate airway management are lifesaving 6.