Diminished capacity of opsonization and immune complex solubilization, and detection of anti-C1q antibodies in sera from patients with hereditary angioedema
Diminished capacity of opsonization and immune complex solubilization, and detection of anti-C1q antibodies in sera from patients with hereditary angioedema
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DOI：
10.1016/j.alit.2017.03.008
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发表时间：
2017-10-01
影响因子：
6.8
通讯作者：
Suzuki, Yusuke
中科院分区：
文献类型：
--
作者：
Honda, Daisuke;Ohsawa, Isao;Suzuki, Yusuke
背景：遗传性血管性水肿（HAE）是一种由C1酯酶抑制剂缺乏引起的常染色体显性遗传病。HAE的症状包括水肿，这可能导致窒息。一些HAE患者表现出免疫学异常，这可能会妨碍准确诊断。低水平的补体成分是HAE的特征，并且在其他情况下被认为减少凋亡细胞和免疫复合物（IC）的消除。因此，我们的目的是通过实验来澄清免疫异常的机制，使用血清从HAE patients.Methods：血清样本从18例HAE患者收集时，从血管性水肿攻击和比较正常的人汇集血清（NHPS）从20名健康志愿者。调理作用被测量为从与血清一起孵育的THP-1细胞分化的巨噬细胞对凋亡Jurkat细胞的吞噬率。通过定量从由过氧化物酶和抗过氧化物酶抗体组成的合成IC释放的过氧化物酶来分析IC在血清中的溶解。结果：12例患者血清学免疫学异常，其中12例患者血清学免疫学异常，12例患者血清学免疫学异常。来自患有HAE的每个患者的血清样品中的调理素化低于NHPS中的调理素化（分别类似于20%对70%）。HAE患者血清中IC溶解率低于NHPS。部分患者血清抗C1q抗体水平较高，血清IC水平升高。结论：HAE患者血清中存在抗C1q抗体，调理作用和IC溶解能力较低。这可能与免疫学异常有关，应进一步研究以促进HAE的准确诊断。Copyright（C）2017，日本变态反应学会.制作和主办：Elsevier B.V.
Background: Hereditary angioedema (HAE) is an autosomal dominant disease caused by deficiency of C1 esterase inhibitor. Symptoms of HAE include edema, which can potentially cause suffocation. Some patients with HAE exhibit immunological abnormalities, which could prevent an accurate diagnosis. Low levels of complement components are characteristic of HAE and in other settings are thought to reduce elimination of apoptotic cells and immune complex (IC). Thus, we aimed to experimentally clarify the mechanism of immunological abnormalities using sera from HAE patients.Methods: Serum samples from 18 patients with HAE were collected when free from angioedema attack and compared with normal human pooled sera (NHPS) from 20 healthy volunteers. Opsonization was measured as the rate of phagocytosis of apoptotic Jurkat cells by macrophages differentiated from THP-1 cells incubated with serum. IC solubilization in serum was analyzed by quantifying peroxidase released from a synthetic IC composed of peroxidase and anti-peroxidase antibodies. Anti-C1q antibody levels were detected using an enzyme-linked immunosorbent assay.Results: Serological immunological abnormalities were detected in 12 patients. Opsonization in serum samples from each patient with HAE was lower than that in NHPS (similar to 20% versus 70%, respectively). The rate of IC solubilization was lower in serum from HAE patients than NHPS. Some patients had high serum anti-C1q antibody levels with increased serum IC levels.Conclusions: Sera from patients with HAE exhibit anti-C1q antibodies, with a lower capacity for opsonization and IC solubilization. This may be associated with immunological abnormalities and should be investigated further to facilitate accurate diagnosis of HAE. Copyright (C) 2017, Japanese Society of Allergology. Production and hosting by Elsevier B.V.