Th2 predominance and decreased NK cells in patients with hereditary angioedema
BackgroundIn 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...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2025-05-01
|
| Series: | Frontiers in Immunology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1536128/full |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1850136149615443968 |
|---|---|
| author | Linda Sundler Björkman Evelina Elmér Evelina Elmér Arne Egesten Lillemor Skattum Lillemor Skattum |
| author_facet | Linda Sundler Björkman Evelina Elmér Evelina Elmér Arne Egesten Lillemor Skattum Lillemor Skattum |
| author_sort | Linda Sundler Björkman |
| collection | DOAJ |
| description | BackgroundIn 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.ObjectiveTo investigate lymphocyte subpopulations in relation to disease activity and complement activation in HAE-C1INH patients and matched controls.MethodsFlow cytometry of peripheral blood lymphocyte populations, measurements of complement and complement fragments, and collection of clinical data.ResultsNK 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.ConclusionsBlood 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. |
| format | Article |
| id | doaj-art-702489aba0154e85b8d0bf3e6d870adc |
| institution | OA Journals |
| issn | 1664-3224 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj-art-702489aba0154e85b8d0bf3e6d870adc2025-08-20T02:31:12ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-05-011610.3389/fimmu.2025.15361281536128Th2 predominance and decreased NK cells in patients with hereditary angioedemaLinda Sundler Björkman0Evelina Elmér1Evelina Elmér2Arne Egesten3Lillemor Skattum4Lillemor Skattum5Respiratory Medicine, Allergology & Palliative Medicine, Department of Clinical Sciences Lund, Lund University and Skåne University Hospital, Lund, SwedenDivision of Transfusion Medicine, Department of Laboratory Medicine, Lund University, Lund, SwedenDepartment of Clinical Immunology and Transfusion Medicine, Skåne University Hospital, Lund, SwedenRespiratory Medicine, Allergology & Palliative Medicine, Department of Clinical Sciences Lund, Lund University and Skåne University Hospital, Lund, SwedenDepartment of Clinical Immunology and Transfusion Medicine, Skåne University Hospital, Lund, SwedenDivision of Microbiology, Immunology and Glycobiology, Department of Laboratory Medicine, Lund University, Lund, SwedenBackgroundIn 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.ObjectiveTo investigate lymphocyte subpopulations in relation to disease activity and complement activation in HAE-C1INH patients and matched controls.MethodsFlow cytometry of peripheral blood lymphocyte populations, measurements of complement and complement fragments, and collection of clinical data.ResultsNK 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.ConclusionsBlood 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.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1536128/fullhereditary angioedemaadaptive immunityNK cellsT helper 2 cellscomplement activation |
| spellingShingle | Linda Sundler Björkman Evelina Elmér Evelina Elmér Arne Egesten Lillemor Skattum Lillemor Skattum Th2 predominance and decreased NK cells in patients with hereditary angioedema Frontiers in Immunology hereditary angioedema adaptive immunity NK cells T helper 2 cells complement activation |
| title | Th2 predominance and decreased NK cells in patients with hereditary angioedema |
| title_full | Th2 predominance and decreased NK cells in patients with hereditary angioedema |
| title_fullStr | Th2 predominance and decreased NK cells in patients with hereditary angioedema |
| title_full_unstemmed | Th2 predominance and decreased NK cells in patients with hereditary angioedema |
| title_short | Th2 predominance and decreased NK cells in patients with hereditary angioedema |
| title_sort | th2 predominance and decreased nk cells in patients with hereditary angioedema |
| topic | hereditary angioedema adaptive immunity NK cells T helper 2 cells complement activation |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1536128/full |
| work_keys_str_mv | AT lindasundlerbjorkman th2predominanceanddecreasednkcellsinpatientswithhereditaryangioedema AT evelinaelmer th2predominanceanddecreasednkcellsinpatientswithhereditaryangioedema AT evelinaelmer th2predominanceanddecreasednkcellsinpatientswithhereditaryangioedema AT arneegesten th2predominanceanddecreasednkcellsinpatientswithhereditaryangioedema AT lillemorskattum th2predominanceanddecreasednkcellsinpatientswithhereditaryangioedema AT lillemorskattum th2predominanceanddecreasednkcellsinpatientswithhereditaryangioedema |