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Th2 predominance and decreased NK cells in patients with hereditary angioedema

Sundler Björkman, Linda LU orcid ; Elmér, Evelina LU orcid ; Egesten, Arne LU and Skattum, Lillemor LU (2025) In Frontiers in Immunology 16.
Abstract

Background: In this study we included patients with hereditary angioedema (HAE) caused by decreased levels 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. Objective: 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... (More)

Background: In this study we included patients with hereditary angioedema (HAE) caused by decreased levels 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. Objective: 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.

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author
; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
adaptive immunity, complement activation, hereditary angioedema, NK cells, T helper 2 cells
in
Frontiers in Immunology
volume
16
article number
1536128
publisher
Frontiers Media S. A.
external identifiers
  • scopus:105006803005
  • pmid:40438097
ISSN
1664-3224
DOI
10.3389/fimmu.2025.1536128
language
English
LU publication?
yes
id
0823d2a3-41c4-40b7-ba0d-a74235f6a2bc
date added to LUP
2025-08-19 11:37:44
date last changed
2025-09-30 15:17:37
@article{0823d2a3-41c4-40b7-ba0d-a74235f6a2bc,
  abstract     = {{<p>Background: In this study we included patients with hereditary angioedema (HAE) caused by decreased levels 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. Objective: 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.</p>}},
  author       = {{Sundler Björkman, Linda and Elmér, Evelina and Egesten, Arne and Skattum, Lillemor}},
  issn         = {{1664-3224}},
  keywords     = {{adaptive immunity; complement activation; hereditary angioedema; NK cells; T helper 2 cells}},
  language     = {{eng}},
  publisher    = {{Frontiers Media S. A.}},
  series       = {{Frontiers in Immunology}},
  title        = {{Th2 predominance and decreased NK cells in patients with hereditary angioedema}},
  url          = {{http://dx.doi.org/10.3389/fimmu.2025.1536128}},
  doi          = {{10.3389/fimmu.2025.1536128}},
  volume       = {{16}},
  year         = {{2025}},
}