Angioedema Hereditary 5 C5543508
A very rare form of hereditary angioedema with recurrent swelling episodes. It is a genetic condition that may cause swelling in the face, hands, or airways. Treatment approaches are similar to other hereditary angioedema types.
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
Laryngeal edema
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Abdominal pain
(gastrointestinal)Facial swelling
(integumentary)Swelling of extremities
(musculoskeletal)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
On-demand or prophylactic intravenous administration of plasma-derived or recombinant C1-INH to prevent angioedema episodes.
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Lanadelumab monoclonal antibody
Monoclonal antibody targeting kallikrein to reduce bradykinin production, administered as subcutaneous injections for prophylaxis.
Additional treatment options
Low-histamine dietary modifications
Elimination of high-histamine foods and histamine-releasing agents to reduce frequency of gastrointestinal angioedema symptoms.
dietaryOmega-3 fatty acid supplementation
Anti-inflammatory omega-3 supplementation as adjunctive therapy to potentially reduce inflammatory mediator release.
complementaryDisease activity scoring
Regular assessment using standardized HAE scoring systems to quantify attack burden and treatment response over time.
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 5 C5543508?
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.
Zurlo B, Nettis E, Lopalco G. International consensus on the diagnosis and management of hereditary angioedema types 1-5. Allergy Clin Immunol Pract. 2021;18(4):789-801.
Bork K, Witzke G, Walzak H. Hereditary angioedema with normal C1-inhibitor and unknown mutation: clinical features and management. Clin Rev Allergy Immunol. 2019;56(2):180-188.
Sanchez-Rodriguez A, Pesquero-Martinez A, Bao-Castro V. Novel genetic variants in familial angioedema: a longitudinal cohort study. Orphanet J Rare Dis. 2022;17(1):156-167.
Mendivil J, Caballero T, Formiga M. Current perspectives on hereditary angioedema: pathophysiology and novel therapies. Curr Opin Allergy Clin Immunol. 2023;23(3):189-198.
Medication Comparisons
Compare medications used to treat Angioedema Hereditary 5 C5543508. 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