ORIGINAL RESEARCH article
Front. Immunol.
Sec. Immunological Tolerance and Regulation
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1536128
Th2 predominance and decreased NK cells in patients with hereditary angioedema
Provisionally accepted
- 1Division of Respiratory Medicine, Allergology and Palliative Medicine, Department of Clinical Sciences Lund, Lund University, Lund, Sweden
- 2Department of Respiratory Medicine and Allergy, Skåne University Hospital, Lund, Sweden
- 3Department of Laboratory Medicine, Transfusion Medicine, Lund University, Lund, Sweden
- 4Department of Clinical Immunology and Transfusion Medicine, Region Skåne, Lund, Sweden
- 5Division of Microbiology, Immunology and Glycobiology, Department of Laboratory Medicine, Faculty of Medicine, Lund University, Lund, Sweden
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Background In this study we included patients with hHereditary angioedema (HAE) is caused by decreased levels or defective function of C1 inhibitor (HAE-C1INH). An increased risk of autoimmune disorders, particularly systemic lupus erythematosus (SLE), has been reported in HAE-C1INH. This suggests that complement consumption affects adaptive immunity.To investigate lymphocyte subpopulations in relation to disease activity and complement activation in HAE-C1INH patients and matched controls. Methods Flow cytometry of peripheral blood lymphocyte populations, measurements of complement and complement fragments, and collection of clinical data. Results NK cell counts were lower in HAE-C1INH patients, and their frequencies were related to disease activity. The T helper (Th) cell balance was skewed towards more Th2 cells and less Th1 cells in HAE-C1INH patients compared to controls. There were also lower frequencies of class-switched B cells and plasmablasts in patients. Levels of C4, and the complement activation fragment C3d were related to disease activity. Conclusions Blood lymphocyte populations are altered in HAE-C1INH, a finding which may be of pathophysiological importance considering the increased risks of both autoimmunity and allergy associated with HAE-C1INH.
Keywords: hereditary angioedema, Adaptive Immunity, NK cells, T helper 2 cells, Complement Activation
Received: 28 Nov 2024; Accepted: 14 Apr 2025.
Copyright: © 2025 Sundler Björkman, Elmér, Egesten and Skattum. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Lillemor Skattum, Division of Microbiology, Immunology and Glycobiology, Department of Laboratory Medicine, Faculty of Medicine, Lund University, Lund, 223 62, Sweden
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