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Measurement of C1-Inhibitor function alone is sufficient for diagnosis of hereditary angioedema
Sorena Kiani-Alikhan1,2, Elizabeth Walker3, Alaco Hickey3
1Clinical Immunology, Barts Health NHS Trust, London, UK skiani@nhs.net.
Insights
Testing only C1-inhibitor function is an efficient and sensitive method for diagnosing hereditary angioedema (HAE). This approach achieves 100% sensitivity and reduces diagnostic costs by 45%.
Area of Science:
- Clinical Immunology
- Diagnostic Laboratory Medicine
Background:
- Current guidelines recommend C4, C1-inhibitor level, and function tests for hereditary angioedema (HAE) diagnosis.
- A specialist immunology laboratory serving a high-volume HAE referral center analyzed diagnostic data.
Purpose of the Study:
- To evaluate the efficiency and cost-effectiveness of HAE diagnostic testing protocols.
- To determine if C1-inhibitor function testing alone is sufficient for HAE diagnosis.
Main Methods:
- Retrospective analysis of laboratory data over a 6-month period.
- Comparison of diagnostic yield and cost of different testing strategies.
Main Results:
- Testing only C1-inhibitor function demonstrated 100% sensitivity for HAE diagnosis.
- This streamlined approach reduced laboratory investigation costs by 45%.
Conclusions:
- Single C1-inhibitor function testing is a highly sensitive and cost-effective method for HAE diagnosis.
- This strategy simplifies laboratory workflows and reduces healthcare expenses.
Abstract:
The World Allergy Organisiation/European Academy of Allergy and Clinical Immunology (WAO/EAACI) 2017/2018 guidelines recommend measuring complement4 levels, followed by C1-inhibitor level and function for diagnosis of hereditary angioedema (HAE). We analysed 6 months' worth of data generated in our laboratory which is a specialist regional immunology service and also provides laboratory service for the Barts Health immunology department, which is a GA2LEN/HAEi-Angioedema Centre of Excellence and Reference (ACARE) and hence, investigates a large number of patients for HAE. We found that an efficient and sensitive approach for laboratory diagnosis of HAE is to only test the C1-inhibitor function. This approach had a sensitivity of 100% and reduced the cost of laboratory investigations for HAE diagnosis by 45%.
