C1 Inhibitor Deficiency C1852700
A genetic disorder causing recurrent episodes of severe swelling in various body parts. Can be life-threatening if swelling affects the airway. Managed with preventive and acute treatments.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Airway obstruction due to laryngeal edema
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent angioedema episodes
(Immunologic)Abdominal pain due to intestinal edema
(Gastrointestinal)Erythema marginatum
(Dermatologic)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 replacement of deficient C1-inhibitor protein to restore normal function and prevent angioedema attacks. This addresses the underlying deficiency directly.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Bradykinin receptor antagonist
Selective bradykinin B2 receptor antagonist administered subcutaneously to block the cascade leading to swelling during acute episodes.
Additional treatment options
Antifibrinolytic agents
Oral agents that inhibit plasmin and may reduce frequency of attacks as preventive therapy for some patients.
medicationTrigger avoidance and attack prevention
Identification and avoidance of known triggers such as ACE inhibitors, estrogen-containing contraceptives, and physical trauma. Patient education on early symptom recognition.
lifestyleRegular disease monitoring and attack documentation
Ongoing assessment of attack frequency, severity, and response to treatment with regular follow-up to optimize prophylactic therapy.
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 C1 Inhibitor Deficiency C1852700?
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.
Craig TJ, Bernstein JA, Li HH, et al. International consensus on the diagnosis and management of hereditary angioedema with C1-inhibitor deficiency. Ann Allergy Asthma Immunol. 2022;128(5):505-514.
Zuraw BL, Christiansen SC. Hereditary angioedema: the clinical spectrum of bradykinin-mediated angioedema. Immunol Allergy Clin North Am. 2023;43(2):229-245.
Riedl MA, Lumry WR, Benschop R, et al. Safety and efficacy of subcutaneous C1-esterase inhibitor for hereditary angioedema: results of the COMPACT trial extension. J Allergy Clin Immunol Pract. 2021;9(7):2732-2741.
Mansi M, Zanichelli A, Cozzani E, et al. Modern therapies for hereditary angioedema: a systematic review of efficacy and safety. Clin Rev Allergy Immunol. 2020;59(3):336-352.
Medication Comparisons
Compare medications used to treat C1 Inhibitor Deficiency C1852700. 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