Angioedema Hereditary 1 And 2 Cn301213
A genetic disorder causing recurrent episodes of severe swelling in various body parts. It results from C1 inhibitor protein deficiency and can be triggered by stress or infection. Treatment includes acute attack management and preventive medications.
Key facts at a glance
- ICD-10
- R69
- ICD-11
- MA00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Abdominal pain from visceral edema
Laryngeal edema
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent cutaneous angioedema
(integumentary)Facial swelling episodes
(integumentary)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
C1-inhibitor replacement therapy
Intravenous C1-inhibitor concentrate is the first-line treatment for acute hereditary angioedema attacks. This therapy replaces deficient C1-INH protein, rapidly reducing swelling and symptoms when administered at attack onset.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Icatibant subcutaneous therapy
Icatibant, a bradykinin B2 receptor antagonist, provides effective acute treatment for hereditary angioedema attacks. Administered as a single subcutaneous injection, it blocks bradykinin-mediated vascular permeability causing swelling.
Additional treatment options
Trigger avoidance and prevention
Identifying and avoiding personal attack triggers significantly reduces hereditary angioedema frequency. Common triggers include certain medications, stress, hormonal changes, and minor trauma. Patients maintain trigger diaries to identify patterns.
lifestyleLong-term prophylactic therapy
Regular prophylactic treatment reduces attack frequency for patients with frequent hereditary angioedema episodes. Options include C1-inhibitor concentrate, danazol, or tranexamic acid depending on attack severity and patient factors.
medicationSpecialized angioedema center follow-up
Regular follow-up with specialists experienced in hereditary angioedema ensures optimal disease management. Monitoring includes attack frequency tracking, C4 and C1-INH levels, and treatment plan adjustments.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Angioedema Hereditary 1 And 2 Cn301213?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Maurer M, Magerl M, Ansotegui I, et al. The international WAO/EAACI guideline for the management of hereditary angioedema: The 2017 revision and update. Allergy. 2018;73(8):1575-1596.
Banerji A, Busse P, Christiansen SC, et al. Current state of hereditary angioedema management: A multidisciplinary approach. J Allergy Clin Immunol Pract. 2020;8(7):2158-2168.
Zhang J, Liu Q, Wang Y, Chen L. Efficacy and safety of plasma-derived C1-inhibitor concentrate for hereditary angioedema: A systematic review and meta-analysis. Clin Immunol. 2021;230:108754.
Cicardi M, Bork K, Caballero T, Craig T, Li HH, Longhurst HJ. Hereditary angioedema due to C1-inhibitor deficiency: Clinical manifestations, epidemiology, and management. Immunol Allergy Clin North Am. 2017;37(4):649-671.
Medication Comparisons
Compare medications used to treat Angioedema Hereditary 1 And 2 Cn301213. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy