肝功能指标异常及血清IgG4升高患者的临床特点及潜在机制

IF 2.7 4区 医学 Q2 Medicine Canadian Journal of Gastroenterology and Hepatology Pub Date : 2022-08-25 eCollection Date: 2022-01-01 DOI:10.1155/2022/7194826
Jing Wang, Yue Zhang, Dandan Jiang, Lu Zhou, Bangmao Wang
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引用次数: 0

摘要

目的:分析肝功能指标异常、血清IgG4水平升高患者的病因分型及临床特点,探讨肝内滤泡性辅助性T细胞(Tfh)浸润及血清IL-21的影响。方法:对136例肝损伤患者的临床资料(年龄、性别、既往史、临床表现、实验室检查、影像学、诊断、治疗)及病因进行分析。我们比较了19例血清IgG4水平升高的AIH(自身免疫性肝炎)患者与20例同时入院的血清IgG4水平正常的AIH患者的一般情况、临床特征和实验室检查。将5例AIH血清IgG4水平升高患者与5例IgG4水平正常的AIH患者进行性别、年龄、肝功能匹配,免疫荧光染色检测两组患者肝活检组织中Tfh的浸润情况。选择5例血清IgG4水平升高的AIH患者,采用酶联免疫吸附试验(ELISA)测定血清白细胞介素-21 (IL-21)水平,17例血清IgG4正常的AIH患者按性别、年龄、肝功能指标进行配对,29例按性别、年龄匹配的身体健康者作为对照组。测定糖皮质激素治疗前后患者IgG4-RD及肝功能异常的变化。结果:136例肝功能指标异常、血清IgG4水平升高的患者均诊断为不同病因的肝病。igg4相关疾病最为常见,其次是AIH和恶性肿瘤。肝功能指标异常伴血清IgG4增高,以谷氨酰转移酶(GGT)升高最为常见。血清IgG4升高的AIH组肝内Tfh水平升高。IgG4升高的AIH患者IL-21高于正常IgG4患者和健康对照。肝功能指标异常、IgG4相关疾病患者28例,经糖皮质激素治疗6个月后,ALT、AST、ALKP、GGT、TBil、DBil、IgG、IgG4、IgE均显著降低。结论:多种病因的肝功能指标异常患者血清IgG4均有升高。Tfh浸润和IL-21分泌增加可能与AIH发病机制及血清IgG4升高有关。糖皮质激素治疗对肝功能指标异常和igg4相关疾病的患者有效。对肝功能指标异常、血清IgG4水平升高的患者进行免疫功能评估,有助于本病的诊断和治疗。
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Clinical Characteristics and Potential Mechanisms in Patients with Abnormal Liver Function Indices and Elevated Serum IgG4.

Objective: We analyzed the etiological classification and clinical characteristics of patients with abnormal liver function indices and elevated serum IgG4 levels and investigated the effects of intrahepatic follicular helper T cell (Tfh) infiltration and serum IL-21.

Methods: Clinical data (age, sex, past history, clinical manifestations, laboratory tests, imaging, diagnosis, and treatment) and etiology of liver injury from 136 patients were analyzed. We compared the general condition, clinical characteristics, and laboratory tests of 19 AIH (autoimmune hepatitis) patients with elevated serum IgG4 levels with those of 20 AIH patients with normal serum IgG4 levels admitted at the same time. Five patients with AIH and elevated serum IgG4 levels and five AIH patients with normal IgG4 levels were matched by sex, age, and liver function, and Tfh infiltration in liver biopsy tissues of patients in both groups was determined by immunofluorescence staining. Five AIH patients with elevated serum IgG4 levels were selected for measurement of serum interleukin-21 (IL-21) levels by enzyme-linked immunosorbent assay (ELISA), seventeen AIH patients with normal serum IgG4 were matched by sex, age, and liver function indices, and 29 physically healthy individuals matched by sex and age were selected as the control group. The changes in patients with IgG4-RD and abnormal liver function before and after glucocorticoid treatment were measured.

Results: Patients (136) with abnormal liver function indices and elevated serum IgG4 levels were diagnosed with liver disease of different etiologies. IgG4-related disease was the most frequent, followed by AIH and malignancy. Abnormal liver function indices with high serum IgG4 were most commonly seen as elevated gamma glutamyl transferase (GGT). The AIH group with elevated serum IgG4 had increased intrahepatic levels of Tfh. IL-21 in AIH patients with elevated IgG4 was higher than in patients with normal IgG4 and healthy controls. Patients (n = 28) with abnormal liver function indices and IgG4-related disease received glucocorticoid therapy for six months, and ALT, AST, ALKP, GGT, TBil, DBil, IgG, IgG4, and IgE were significantly lower after treatment.

Conclusions: Elevated serum IgG4 was seen in patients with abnormal liver function indices with diverse causes. Tfh infiltration and increased IL-21 production may be related to the pathogenesis of AIH with elevated serum IgG4. Glucocorticoid therapy is effective in patients with abnormal liver function indices and IgG4-related disease. Assessing immune function in patients with abnormal liver function indices and elevated serum IgG4 levels should facilitate diagnosis and treatment of the disease.

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来源期刊
CiteScore
4.80
自引率
0.00%
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审稿时长
37 weeks
期刊介绍: Canadian Journal of Gastroenterology and Hepatology is a peer-reviewed, open access journal that publishes original research articles, review articles, and clinical studies in all areas of gastroenterology and liver disease - medicine and surgery. The Canadian Journal of Gastroenterology and Hepatology is sponsored by the Canadian Association of Gastroenterology and the Canadian Association for the Study of the Liver.
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