IgG4相关性疾病1例并文献复习  

A case of IgG4-related disease and literature review

作　　者：王东轶 商玮[1] 赵智明[1] 蔡辉[1] WANG Dongyi;SHANG Wei;ZHAO Zhiming;CAI Hui(Department of Integrated Traditional Chinese and Western Medicine,General Hospital of Eastern Military Command,Nanjing,Jiangsu 210000,China)

机构地区：[1]东部战区总医院(原南京军区南京总医院)中西医结合科

出　　处：《安徽医药》2019年第10期2010-2013,I0005,共5页Anhui Medical and Pharmaceutical Journal

摘　　要：目的提高对累及泪腺、淋巴结为主的IgG4相关性疾病(IgG4?RD)的认识和诊治水平。方法分析2017年8月16日东部战区总医院(原南京军区南京总医院)中西医结合科收住的1例IgG4?RD病人的临床表现、实验室检查、组织病理、治疗及预后,并进行文献复习。结果病人男,23岁,因眼干、淋巴结肿大入院,血清IgG4增高,淋巴结活检病理提示符合IgG4相关硬化性疾病改变(IgG4:>50个IgG4^+细胞/高倍视野;IgG4/IgG>50%),诊断IgG4?RD,口服泼尼松15mg/d,3月后复查血清IgG4下降,淋巴结未见明显增大。检索中英文文献,IgG4?RD诊断困难,易与血液系统疾病、反应性疾病相混淆,组织病理仍是“金标准”,在无条件组织活检时血清IgG4亦具有重要意义。治疗上,轻症者可单一糖皮质激素(GC)治疗,重症者在GC的基础上,可联合使用其他传统免疫抑制剂或妥昔单抗(RTX)。结论IgG4?RD诊断需结合临床症状、血清IgG4水平、影像学表现及病理结果,一旦诊断应早期治疗、长期服用GC、小剂量维持,定期复查,谨防复发。对于高度怀疑IgG4?RD的病人可酌情采取早期激素诊断性治疗。Objective To promote the cognition,diagnosis and treatment of IgG4?related disease(IgG4?RD)involving lacrimal gland and lymph gland.Methods Analysis was made of the clinical manifestation,laboratory examination,histopathology,treatment and prognosis of 1 case of IgG4?RD admitted to the Department of Integrated Traditional Chinese and Western Medicine of General Hospital of Eastern Military Command on August 16,2017 and a literature review was conducted.Results A 23?year?old man was admitted to hospital for dryness of eye and lymphadenectasis.Laboratory data showed increased serum level of total IgG4,and histo?pathologic examination of the resected lymph gland indicated the changes of IgG4 related sclerosis(IgG4:>50 IgG4^+cells/high magnification;IgG4/IgG>50%).The diagnosis was an IgG4 related disease.After oral intake of prednisone 15mg/d for 3 months,se?rum IgG4 level was decreased and lymph nodes were not significantly enlarged.Chinese and English literature retrieved showed that the diagnosis of IgG4?RD is difficult and it is likely to be confused with blood system diseases and reactive diseases.Histopathology is still the“gold standard”,and the serum IgG4 is also of great significance in unconditional tissue biopsy.As for the treatment,mild cases can be treated with single glucocorticoid(GC),while severe cases can be treated with GC combined with other tradition?al immunosuppressants or rituximab(RTX).Conclusions Diagnosis of IgG4?RD needs to be based on clinical symptoms,serum IgG4 level,imaging findings and pathological results.Once the disease is diagnosed,it should be treated as early as possible with long?term use,small dose maintenance,regular reexamination to prevent recurrence.For the patients highly suspected to be IgG4?RD,GC can be used early.

关 键 词：IGG4相关性疾病 免疫球蛋白G 浆细胞 泼尼松 诊疗 

分 类 号：R59[医药卫生—内科学]