Hereditary Angioedema With Normal C1inh C1960459
A type of hereditary angioedema occurring despite normal C1-inhibitor levels, more common in women. It causes episodic swelling that may be linked to hormonal factors. Management focuses on preventing and treating swelling episodes with available medications.
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 facial swelling (lips and tongue)
(integumentary)Recurrent limb swelling
(integumentary)Gastrointestinal edema causing abdominal pain
(gastrointestinal)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
Icatibant
First-line acute treatment targeting the bradykinin pathway, which is implicated in normal C1-INH angioedema. Subcutaneous administration allows for convenient and rapid treatment of attacks.
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
Comprehensive Diagnostic Workup
Thorough evaluation to confirm normal C1-INH status and identify specific underlying mutations (F12, ANGPT1, PLG) when possible. Genetic testing may guide treatment selection and family screening.
Additional treatment options
Tranexamic Acid
Antifibrinolytic therapy used for prophylaxis, particularly when attacks are triggered by hormonal changes. May be used alone or in combination with other prophylactic agents.
medicationHormonal Trigger Management
Identification and management of hormonal triggers including oral contraceptives, hormone replacement therapy, and pregnancy-related changes. Working with gynecologist may be beneficial for women.
lifestyleStress Management Techniques
Stress is a recognized trigger for angioedema attacks. Relaxation techniques, mindfulness practices, and stress reduction strategies may help reduce attack frequency.
complementaryWhen 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 With Normal C1inh C1960459?
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.
Zuraw BL, Bernstein JA, Craig TJ, et al. International consensus guidelines for the management of hereditary angioedema with normal C1 inhibitor. J Allergy Clin Immunol Pract. 2022.
Bork K, Wulff K, Witzke G, Hardt J. Hereditary angioedema with normal C1-INH: clinical features and diagnostic challenges. Allergy. 2020.
Aulig C, Aygoren-Pursun E, Magerl M, et al. Open-label study of icatibant for acute attacks in hereditary angioedema with normal C1 inhibitor. Ann Allergy Asthma Immunol. 2021.
Soter SD, Gower RG, Hurewitz DS. Efficacy of plasmaderived C1 inhibitor concentrate in hereditary angioedema with normal C1 inhibitor: a systematic review and meta-analysis. Clin Immunol. 2023.
Medication Comparisons
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy