What are the typical presentation and initial management of Quincke's disease (angioedema)?

Typical Presentation and Initial Management of Quincke's Disease (Angioedema)

Angioedema typically presents as sudden-onset, non-pitting, non-pruritic swelling of the deep dermis and subcutaneous tissues, most commonly affecting the face, lips, tongue, extremities, genitalia, and can involve the gastrointestinal tract and upper airways. 1, 2

Clinical Presentation

Key Features

• Location of swelling: Face, lips, tongue, extremities, genitalia, and potentially life-threatening involvement of the upper airways 1
• Appearance: Non-pitting, non-pruritic edema 3
• Duration: Typically worsens over 24 hours and resolves within 48-72 hours 1
• Prodromal symptoms: May include erythema marginatum (non-urticarial rash), localized tingling, or skin tightness 1

Types of Angioedema and Distinguishing Features

1. Histamine-mediated angioedema:

   • Usually presents with urticaria (hives)
   • Often associated with allergen exposure
   • Responds to antihistamines and corticosteroids 2

2. Bradykinin-mediated angioedema (including hereditary forms):

   • No associated urticaria
   • Does not respond to antihistamines or corticosteroids
   • Includes hereditary angioedema (HAE), acquired C1 inhibitor deficiency, and ACE inhibitor-induced angioedema 2, 3

Specific Presentations by Type

Hereditary Angioedema (HAE)

• Onset often in childhood, worsening around puberty 1
• Autosomal dominant inheritance pattern (75% have family history) 1
• Episodic attacks (not continuous daily swelling) 1
• Abdominal attacks cause severe pain, nausea, vomiting, and fluid sequestration 1
• Laryngeal attacks can be life-threatening (historically 30% mortality) 1

ACE Inhibitor-Induced Angioedema

• Accounts for 30-40% of angioedema cases seen in US emergency departments 3
• Higher risk in African Americans, people over 65, women, and those with smoking history 2
• Can cause significant macroglossia (tongue swelling) 4

Initial Management

Emergency Assessment

1. Airway evaluation: Immediately assess for signs of airway compromise 2
2. Monitor oxygen saturation: Maintain levels above 92% 2
3. Consider early elective intubation: If signs of impending airway closure develop 2
4. Be prepared for emergency tracheostomy: If intubation fails 2

Treatment Based on Type

For Histamine-Mediated Angioedema:

• First-line: H1 antihistamines (cetirizine, desloratadine, fexofenadine) 2
• Add-on: H2 antihistamines (famotidine) for better control 2
• For severe cases: Corticosteroids (methylprednisolone 125 mg IV) 2
• For anaphylaxis: Epinephrine 2, 5

For Bradykinin-Mediated Angioedema (including HAE):

• First-line for acute attacks:
  • Plasma-derived C1 esterase inhibitor (20 IU/kg IV) 2
  • Icatibant 30 mg subcutaneously (may repeat at 6-hour intervals, max 3 injections/24 hours) 2, 6
  • Ecallantide (plasma kallikrein inhibitor) 2
• Note: Antihistamines, corticosteroids, and epinephrine are NOT effective 2

For ACE Inhibitor-Induced Angioedema:

• First step: Discontinue the ACE inhibitor 4
• Supportive care: Antihistamines and corticosteroids may be tried but have limited efficacy 3
• Airway management: Critical in severe cases 4

Special Considerations

• Abdominal attacks: May require narcotic pain medication, antiemetics, and aggressive hydration 1
• Pregnancy: Avoid attenuated androgens; tranexamic acid may be better tolerated 2
• Long-term prophylaxis options for recurrent HAE:
  • Plasma-derived C1INH replacement
  • Lanadelumab (monoclonal antibody against plasma kallikrein)
  • Attenuated androgens (danazol 100-600 mg daily)
  • Antifibrinolytic agents (tranexamic acid) 2

Common Pitfalls and Caveats

1. Misdiagnosis: Abdominal attacks of HAE are frequently misdiagnosed, leading to unnecessary surgical interventions 1
2. Ineffective treatments: Using antihistamines and corticosteroids for bradykinin-mediated angioedema is ineffective 2
3. Delayed diagnosis: Average delay in diagnosis of HAE is significant; early identification is crucial 1
4. Medication triggers: Always review medication list for ACE inhibitors as potential causes 5
5. Estrogen avoidance: Women with HAE should avoid estrogen-containing contraceptives 2

Remember that angioedema involving the upper airways can be rapidly progressive and potentially fatal, requiring immediate intervention and close monitoring in a facility capable of emergency airway management.