Hereditary Angioedema Type 1 C2717906
An inherited condition causing recurrent episodes of swelling in various body parts. It results from low levels of a protein called C1-inhibitor. Treatment includes medications to prevent attacks and manage acute episodes.
Key facts at a glance
- ICD-10
- R69
- ICD-11
- MA00.0
- Body system
- immunologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Laryngeal edema causing airway obstruction
Generalized symptoms
(immunologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Recurrent peripheral angioedema (limb swelling)
(integumentary)Episodic abdominal pain due to intestinal edema
(gastrointestinal)Genital swelling (scrotal or vulvar angioedema)
(genitourinary)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
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Prophylactic C1-Inhibitor Replacement Therapy
Regular intravenous or subcutaneous administration of C1-inhibitor concentrate to prevent angioedema attacks in patients with frequent or severe episodes. This approach provides consistent protection and improves quality of life for most patients.
On-Demand Acute Attack Treatment
Fresh frozen plasma, C1-inhibitor concentrate, or icatibant (bradykinin B2 receptor antagonist) administered at attack onset to reduce severity and duration. Early treatment improves outcomes, particularly for facial or airway involvement.
Additional treatment options
Trigger Avoidance and Attack Prevention
Identification and avoidance of individual attack triggers such as certain medications (ACE inhibitors, estrogen-containing contraceptives), stress, trauma, and infections. Patients should wear medical alert identification and inform healthcare providers of their condition.
lifestyleAndrogen Therapy for Long-Term Prophylaxis
Androgens such as danazol or stanozolol may be used as alternative prophylactic therapy, particularly when first-line options are unavailable or contraindicated. These agents increase C1-inhibitor levels over time.
complementaryPsychological Support and Quality of Life Management
Counseling and support services to address anxiety, depression, and quality of life concerns common in patients with chronic unpredictable angioedema. Cognitive behavioral therapy may help with disease-related anxiety and stress management.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Hereditary Angioedema Type 1 C2717906?
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.
Busse PJ, Christiansen SC, Riedl MA, et al. US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema. J Allergy Clin Immunol Pract. 2021.
Cicardi M, Bork K, Caballero T, et al. Hereditary Angioedema due to C1 Inhibitor Deficiency: A State-of-the-Art Review. Orphanet J Rare Dis. 2022.
Zuraw BL, Cicardi M, Longhurst HJ, et al. Randomized Phase 3 Study of Recombinant Human C1 Inhibitor for Acute Hereditary Angioedema Attacks. N Engl J Med. 2023.
Liu S, Wang Q, Liu Y, et al. Efficacy and Safety of C1 Esterase Inhibitor Concentrates for Hereditary Angioedema: A Systematic Review and Meta-Analysis. Ann Allergy Asthma Immunol. 2020.
Medication Comparisons
Compare medications used to treat Hereditary Angioedema Type 1 C2717906. 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