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遗传性血管性水肿患者中Th2优势及自然杀伤细胞减少

Th2 predominance and decreased NK cells in patients with hereditary angioedema.

作者信息

Sundler Björkman Linda, Elmér Evelina, Egesten Arne, Skattum Lillemor

机构信息

Respiratory Medicine, Allergology & Palliative Medicine, Department of Clinical Sciences Lund, Lund University and Skåne University Hospital, Lund, Sweden.

Division of Transfusion Medicine, Department of Laboratory Medicine, Lund University, Lund, Sweden.

出版信息

Front Immunol. 2025 May 14;16:1536128. doi: 10.3389/fimmu.2025.1536128. eCollection 2025.

DOI:10.3389/fimmu.2025.1536128
PMID:40438097
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12116338/
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 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.

摘要

背景

在本研究中，我们纳入了因C1抑制剂水平降低导致的遗传性血管性水肿（HAE-C1INH）患者。已有报道称，HAE-C1INH患者患自身免疫性疾病的风险增加，尤其是系统性红斑狼疮（SLE）。这表明补体消耗会影响适应性免疫。

目的

研究HAE-C1INH患者及匹配对照中与疾病活动和补体激活相关的淋巴细胞亚群。

方法

对外周血淋巴细胞群体进行流式细胞术检测、补体及补体片段测量，并收集临床数据。

结果

HAE-C1INH患者的自然杀伤（NK）细胞计数较低，其频率与疾病活动相关。与对照组相比，HAE-C1INH患者的辅助性T（Th）细胞平衡向更多Th2细胞和更少Th1细胞倾斜。患者中类别转换B细胞和浆母细胞的频率也较低。C4水平和补体激活片段C3d与疾病活动相关。

结论

HAE-C1INH患者的血液淋巴细胞群体发生改变，考虑到HAE-C1INH患者自身免疫和过敏风险增加，这一发现可能具有病理生理学意义。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/00cc66454b58/fimmu-16-1536128-g009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/6878f173347f/fimmu-16-1536128-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/d12f05e5385f/fimmu-16-1536128-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/fec12a8db0ab/fimmu-16-1536128-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/2d68eb288ea7/fimmu-16-1536128-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/c5d150b8157f/fimmu-16-1536128-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/cc0d7ef1aa15/fimmu-16-1536128-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/dd4a0bc69b23/fimmu-16-1536128-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/bca97369159e/fimmu-16-1536128-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/00cc66454b58/fimmu-16-1536128-g009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/6878f173347f/fimmu-16-1536128-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/d12f05e5385f/fimmu-16-1536128-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/fec12a8db0ab/fimmu-16-1536128-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/2d68eb288ea7/fimmu-16-1536128-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/c5d150b8157f/fimmu-16-1536128-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/cc0d7ef1aa15/fimmu-16-1536128-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/dd4a0bc69b23/fimmu-16-1536128-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/bca97369159e/fimmu-16-1536128-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b9d/12116338/00cc66454b58/fimmu-16-1536128-g009.jpg

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本文引用的文献

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