What is the natural history of Angioedema (Quincke's edema)?

From the Guidelines

Angioedema, also known as Quincke's edema, is a condition characterized by rapid onset swelling that can affect various body parts, with a natural history that varies depending on whether it's hereditary or acquired, and its management is crucial to prevent life-threatening complications, especially with laryngeal involvement, as evidenced by studies such as 1 and 1. The natural history of angioedema can be divided into hereditary and acquired forms, with hereditary angioedema (HAE) being a chronic disorder that may occasionally lead to mortality due to laryngeal edema, as noted in 1.

Key Characteristics of Angioedema

• Episodes usually develop over minutes to hours, peak within 12-24 hours, and resolve spontaneously within 24-72 hours without treatment, as described in 1.
• In hereditary angioedema, attacks may occur every few weeks or months with varying severity, often triggered by trauma, stress, or hormonal changes.
• Acquired forms may be allergic (resolving within hours to days) or medication-induced (particularly from ACE inhibitors, which can persist until the medication is discontinued).

Importance of Proper Management

Without proper management, life-threatening complications can occur, especially with laryngeal involvement, highlighting the need for prompt treatment of acute episodes and appropriate prophylaxis for recurrent cases, as emphasized in 1.

Treatment Options

For hereditary forms, C1 esterase inhibitor replacement, kallikrein inhibitors, or bradykinin receptor antagonists may be needed, as these have been shown to be safe and efficacious for the treatment of acute HAE attacks, as noted in 1.

Emergency Preparedness

The unpredictable nature of attacks makes it essential for patients to have an emergency action plan and carry rescue medication such as epinephrine auto-injectors for allergic forms or specific medications for hereditary types, as suggested in 1 and 1. Some key points to consider in the management of angioedema include:

• On-demand treatment is most effective when administered as early as possible in an attack, as noted in 1.
• Standard angioedema treatment modalities, such as epinephrine, corticosteroids, or antihistamines, do not have a significant effect on the swelling in patients with HAE, as stated in 1 and 1.
• Fresh frozen plasma has been used to treat acute angioedema attacks, but it can sometimes cause a sudden worsening of symptoms and carries the inherent risk of viral transmission, as described in 1.

From the Research

Definition and Forms of Angioedema

• 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.
• There are different forms of angioedema, including hereditary angioedema (HAE) and non-hereditary forms, such as Quincke's disease 3.

Clinical Manifestations

• Clinical manifestations of angioedema include a non-erythematous swollen uvula, fullness in the throat, and signs of airway obstruction 3.
• Patients with angioedema may present to the emergency department with life-threatening conditions, such as laryngeal edema, or with symptoms mimicking an acute abdomen 2.

Diagnosis and Treatment

• Diagnosis of angioedema is primarily clinical, but laboratory tests can be used to confirm the diagnosis and differentiate between histaminergic and non-histaminergic angioedema 2.
• Treatment of angioedema depends on the underlying cause, with allergic histaminergic angioedema responding to standard therapies such as epinephrine, glucocorticoids, and antihistamines, while non-histaminergic angioedema is often resistant to these drugs 2.
• Therapeutic options for angioedema due to C1-inhibitor deficiencies include C1-inhibitor concentrates, icatibant, and ecallantide, while fresh frozen plasma can be considered if these drugs are not available 4, 2.

Natural History and Prognosis

• Hereditary angioedema is extremely rare and clinically characterized by recurrent and self-limiting skin, intestinal, and life-threatening laryngeal edema 5.
• The natural history of angioedema can be unpredictable, with some patients experiencing recurrent episodes even after discontinuation of the causative drug, such as ACE inhibitors 6.
• The prognosis of angioedema depends on the underlying cause and the promptness and effectiveness of treatment, with careful observation and appropriate airway management being crucial in preventing serious complications 5, 6.