From the Guidelines
Diagnosis and Treatment of Angioedema (Angioneurotic Edema) The diagnosis and treatment of Angioedema, also known as Angioneurotic Edema, should focus on identifying the underlying cause, such as Hereditary Angioedema (HAE), Acquired C1 Inhibitor Deficiency, or Angiotensin-Converting Enzyme (ACE) Inhibitor-Associated Angioedema, and managing the condition with on-demand HAE-specific agents, such as C1INH concentrates, plasma kallikrein inhibitors, or bradykinin B2 receptor antagonists 1, 2.
Diagnosis
The diagnosis of HAE involves laboratory findings, including low C1INH antigenic and functional levels, decreased C4 levels, and normal C1q levels 1. Two forms of HAE, type I and type II, can be diagnosed based on these laboratory findings. Type I HAE presents with low C1INH antigenic and functional levels, whereas type II HAE presents with normal C1INH antigenic levels but decreased C1INH functional levels.
Treatment
The treatment of HAE attacks involves on-demand therapy with C1INH concentrates, plasma kallikrein inhibitors, or bradykinin B2 receptor antagonists, which have been shown to be safe and efficacious in clinical trials 2. Epinephrine, corticosteroids, and antihistamines are not recommended for the treatment of HAE, as they do not have a significant effect on the swelling seen in patients with HAE 3.
- On-demand treatment: C1INH concentrates, plasma kallikrein inhibitors, or bradykinin B2 receptor antagonists, such as icatibant, are effective for the treatment of acute HAE attacks 2.
- Short-term prophylaxis: Fresh frozen plasma, C1INH replacement, or short-term, high-dose anabolic androgen therapy can be used for short-term prophylaxis 1.
- Long-term prophylaxis: Treatment with low-to-moderate doses of anabolic androgens, antifibrinolytic agents, or replacement plasma-derived C1INH can provide effective and relatively safe long-term HAE prophylaxis 1.
Management of Attacks
The management of HAE attacks involves symptomatic treatment based on the region of body swelling. Patients may require narcotic medications for control of pain during abdominal attacks, as well as antiemetics for nausea and vomiting. Aggressive hydration is usually helpful during abdominal attacks. The management of oropharyngeal and laryngeal attacks is primarily focused on maintaining airway patency 2.
- Abdominal attacks: Narcotic medications, antiemetics, and aggressive hydration may be necessary.
- Oropharyngeal and laryngeal attacks: Maintaining airway patency is crucial, and patients should be closely monitored for signs and symptoms of impending airway closure.
From the Research
Diagnosis of Angioedema
- Angioedema is characterized by localized swelling of subcutaneous tissues or mucosa of the upper respiratory or gastrointestinal tract 4
- It can be broadly divided into three main categories: mast cell-mediated, non-mast-cell-mediated (bradykinin-induced), and idiopathic angioedema 5
- The diagnosis of angioedema involves identifying the underlying cause, which can be hereditary, acquired, or drug-induced 6, 7
Treatment of Angioedema
- The mainstays of management for angioedema are: (1) to avoid specific triggers (if possible and where known) and (2) treatment with medication (if indicated) 5
- Securing the airway remains the most important intervention, followed by administration of both established and more novel pharmacologic interventions based on disease pathology 6
- For hereditary angioedema (HAE), there are specifically licensed treatments that can be used for the management of attacks, or for prophylaxis in order to prevent attacks 5
- The treatment of angioedema depends on the subtype, with histaminergic and non-histaminergic forms requiring different treatments 7
Subtypes of Angioedema
- Mast cell-mediated angioedema (e.g., histamine) 5
- Non-mast-cell-mediated angioedema (bradykinin-induced) 5
- Idiopathic angioedema 5
- Hereditary angioedema (HAE) 5
- Acquired angioedema 6
- Drug-induced angioedema (e.g., angiotensin-converting enzyme inhibitors) 6, 7
Management Approach
- A practical diagnostic and therapeutic approach to the management of angioedema is essential 4
- An evidence-based approach to the diagnosis and suggested management of various subtypes of angioedema is necessary 6
- Cross-disciplinary collaboration is recommended for the acute and prophylactic treatment of angioedema 7
- Enhancing awareness about various angioedema subtypes and their management provides a useful tool for clinical practice 8