以IgE和嗜酸粒细胞升高伴多发淋巴结肿大为主要表现的IgG4相关性疾病1例报告

doi:10.3969/j.issn.1004-583X.2024.01.010

临床荟萃 ›› 2024, Vol. 39 ›› Issue (1): 57-60.doi: 10.3969/j.issn.1004-583X.2024.01.010

以IgE和嗜酸粒细胞升高伴多发淋巴结肿大为主要表现的IgG4相关性疾病1例报告

宋佳亮^a, 蒋英杰^b, 孔瑞娜^c(), 蔡青^c, 高洁^c

a.海军军医大学长海医院康复医学与理疗科,上海 200433
b.海军军医大学长海医院病理科,上海 200433
c.海军军医大学长海医院风湿免疫科,上海 200433

收稿日期:2022-07-12 出版日期:2024-01-20 发布日期:2024-03-22
通讯作者: 孔瑞娜,Email: kongrn725@126.com
基金资助:
国家自然科学基金青年项目成纤维样滑膜细胞外泌体促进软骨细胞铁死亡在骨关节炎中的作用及机制研究(81901583)

IgG4-related diseases characterized by increased IgE level and eosinophil count with multiple lymphadenopathy: A case report

Song Jialiang^a, Jiang Yingjie^b, Kong Ruina^c(), Cai Qing^c, Gao Jie^c

a. Department of Rehabilitation Medicine, Changhai Hospital, Naval Medical University, Shanghai 200433, China
b. Department of Pathology, Changhai Hospital, Naval Medical University, Shanghai 200433, China
c. Department of Rheumatism and Immunology, Changhai Hospital, Naval Medical University, Shanghai 200433, China

Received:2022-07-12 Online:2024-01-20 Published:2024-03-22

摘要/Abstract

摘要：

目的探讨以IgE和嗜酸粒细胞升高伴多发淋巴结肿大为主要表现的IgG4相关性疾病的临床特点和治疗方案。方法回顾性分析1例以IgE和嗜酸粒细胞升高伴多发淋巴结肿大为主要表现的IgG4相关性疾病患者的临床资料、诊疗经过并复习相关文献。结果患者男性,24岁,双侧腹股沟淋巴结肿大2年余入院。结合临床表现、血IgG4检验、病理结果,诊断为IgG4相关性疾病。治疗方案给予强的松20 mg, 1次/日,枸橼酸托法替布片5 mg, 2次/日,根据复查血检验指标和超声结果调整剂量,最终随访患者症状明显改善。结论 lgG4相关性疾病常表现为显著升高的血清IgG4水平和肿块样病灶,合并IgE和嗜酸粒细胞升高的较少见, 但近期研究发现IgE和嗜酸性粒细胞在IgG4相关性疾病中发挥了重要作用。治疗方案以糖皮质激素为一线药物,靶向药物如托法替布可能有潜在的治疗作用,拓展了IgG4相关性疾病治疗的新思路。

Abstract:

Objective To explore the clinical features and treatment of immunoglobulin G4 (IgG4)-related diseases characterized by increased immunoglobulin E (IgE) level and eosinophilic count with multiple lymphadenopathy. Methods The clinical data, diagnosis and treatment process of a patient with IgG4-related disease characterized by increased IgE level and eosinophilic count with multiple lymphadenopathy were retrospective analyzed, and related literatures were reviewed. Results A 24-year-old male patient was admitted with the enlargement of bilateral inguinal lymph nodes for more than 2 years. The clinical manifestations, IgG4 test and pathology results suggested the diagnosis of IgG4-related disease. The therapeutic regimen was 20 mg prednisone orally once a day and 5 mg Tofacitinib citrate tablets orally twice a day. The dosage was adjusted according to the blood test and ultrasound results, and the symptoms of the patient were significantly improved at the final follow-up visit. Conclusion IgG4-related diseases are often characterized by significantly elevated serum IgG4 levels and lump-like lesions. Increased IgE level and eosinophil count are rarely observed in cases of IgG4-related diseases, although their important roles have been recently identified. Glucocorticoids are the first-line drugs, and targeted drugs like Tofacitinib may have potential therapeutic effects on IgG4-related diseases, which expand the new idea of the treatment.

Key words: immunoglobulin G4-related disease, immunoglobulin E, eosinophilia, lymph nodes

中图分类号:

R593.2

宋佳亮, 蒋英杰, 孔瑞娜, 蔡青, 高洁. 以IgE和嗜酸粒细胞升高伴多发淋巴结肿大为主要表现的IgG4相关性疾病1例报告[J]. 临床荟萃, 2024, 39(1): 57-60.

Song Jialiang, Jiang Yingjie, Kong Ruina, Cai Qing, Gao Jie. IgG4-related diseases characterized by increased IgE level and eosinophil count with multiple lymphadenopathy: A case report[J]. Clinical Focus, 2024, 39(1): 57-60.

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链接本文: https://huicui.hebmu.edu.cn/CN/10.3969/j.issn.1004-583X.2024.01.010

https://huicui.hebmu.edu.cn/CN/Y2024/V39/I1/57

图/表 1

图1 右腹股沟淋巴结病理 a~b. 淋巴滤泡显著增生,生发中心散在或灶性分布大量成熟浆细胞,副皮质区部分血管扩张,可见不等量成熟浆细胞,并可见多灶性成熟嗜酸粒细胞浸润(HE,×100;×400);c. IgG4阳性(IHC,×100)

Fig.1 Right inguinal lymph nodes pathology a~b. Lymphoid follicles were significantly hyperplasia, and a large number of mature plasma cells were scattered or focally distributed in the germinal center. Vascular dilatation in part of the paracortical region showed an unequal number of mature plasma cells, and multifocal mature eosinophilic infiltration was observed (HE, ×100; ×400); c. IgG4 positive (IHC, ×100)

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以IgE和嗜酸粒细胞升高伴多发淋巴结肿大为主要表现的IgG4相关性疾病1例报告

IgG4-related diseases characterized by increased IgE level and eosinophil count with multiple lymphadenopathy: A case report

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