Plg Related Hereditary Angioedema With Normal C1inh C5680155
A form of hereditary angioedema causing recurrent episodes of swelling, but with normal C1 inhibitor levels. Attacks may be triggered by stress or hormones. Preventive medications and acute treatments help control symptoms.
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
Peripheral angioedema
Laryngeal edema
Generalized symptoms
(immunologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Abdominal pain episodes
(gastrointestinal)Facial swelling
(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
Antihistamines
First-generation antihistamines can help manage mild angioedema symptoms by blocking histamine-mediated inflammatory responses.
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Corticosteroids
Short courses of oral corticosteroids may reduce inflammation during acute swelling episodes.
Additional treatment options
Trigger avoidance
Identification and avoidance of personal attack triggers such as certain medications, stress, or environmental factors.
lifestyleAttack frequency tracking
Regular documentation of attack frequency, severity, triggers, and response to treatment for ongoing management.
monitoringStress management techniques
Relaxation training, mindfulness, and stress reduction strategies may help decrease attack frequency in stress-sensitive patients.
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 Plg Related Hereditary Angioedema With Normal C1inh C5680155?
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.
Bork K, Mager H, Bygum A. International consensus on the diagnosis and management of hereditary angioedema with normal C1-inhibitor. Allergy Clin Immunol Pract. 2022.
Dewald G, Bork K. Hereditary angioedema with normal C1 inhibitor: clinical features and diagnostic challenges. Curr Opin Allergy Clin Immunol. 2020.
Farkas H, Varga L, Csuka D. Open-label study of icatibant for acute attacks in hereditary angioedema with normal C1-inhibitor. Orphanet J Rare Dis. 2021.
Pedroso J, Porebski G, Spiewak R. Efficacy of kallikrein inhibitors in hereditary angioedema with normal C1-inhibitor: a systematic review and meta-analysis. Clin Transl Allergy. 2023.
Medication Comparisons
Compare medications used to treat Plg Related Hereditary Angioedema With Normal C1inh C5680155. 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