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Measurement of C1-Inhibitor function alone is sufficient for diagnosis of hereditary angioedema
  1. Sorena Kiani-Alikhan1,2,
  2. Elizabeth Walker1,
  3. Alaco Hickey1,
  4. Sofia Grigoriadou1,
  5. Matt Buckland1,
  6. Chris Scott1
  1. 1 Clinical Immunology, Barts Health NHS Trust, London, UK
  2. 2 Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, UK
  1. Correspondence to Dr Sorena Kiani-Alikhan, Clinical Immunology, Barts Health NHS Trust, London E1 1RD, UK; skiani{at}nhs.net

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%.

  • complement system proteins
  • medical laboratory science
  • skin
  • immune system diseases
  • diagnosis

https://doi.org/10.1136/jclinpath-2021-207538

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    Footnotes

    • Handling editor Stephen R A Jolles.

    • Contributors SK-A wrote the article. EW and AH performed the assays. The idea originate from SG and MB. CS pulled and analysed the data.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.