以腹膜及下颌下腺受累的IgG4相关性疾病一例并文献复习  

A case report of IgG4-related disease involving peritoneum and submandibular gland and literature review

作　　者：罗亚丹 查艳[2,3] 杨宇齐 达静静 袁静[2,3] 袁立英 张唐[1] Luo Yadan;Zha Yan;Yang Yuqi;Da Jingjing;Yuan Jing;Yuan Liying;Zhang Tang(Department of Nephrology,the Third Affiliated Hospital of Zunyi Medical University,Zunyi Guizhou 563000,China;Department of Nephrology,Guizhou Provincial People’s Hospital,Guiyang Guizhou 550002,China;Key Laboratory of Diagnosis and Treatment of Pulmonary Immune Diseases,National Health Commission Guizhou Provincial People’s Hospital,Guiyang Guizhou 550002,China)

机构地区：[1]遵义医科大学第三附属医院肾内科,贵州遵义563000 [2]贵州省人民医院肾内科,贵州贵阳550002 [3]国家卫生健康委员会肺脏免疫性疾病诊治重点实验室,贵州贵阳550002

出　　处：《遵义医科大学学报》2023年第11期1106-1110,共5页Journal of Zunyi Medical University

基　　金：贵州省高层次创新人才项目[NO:黔科合平台人才(2018)5636-2];贵州省科技计划项目[NO:黔科合平台人才(2020)2201]。

摘　　要：目的通过回顾1例IgG4相关性疾病同时累及下颌下腺及腹膜的临床特点及诊疗过程,就其临床表现及诊疗进行相关讨论,提高基层医师对IgG4-RD的认识及诊疗。方法分析2019年6月28日遵义医科大学第三附属医院肾内科收治的1例IgG4-RD患者的临床表现、实验室检查、病理检查、诊断、治疗、预后及其文献复习。结果62岁男性患者,因“右侧双J管置入术1月”入院。入院后查血清IgG4增高,腹部计算机断层扫描(CT)提示腹膜后纤维化,下颌下腺病理免疫组化:IgG散在阳性(10~20个/HPF),IgG4/IgG阳性细胞比值约为0.7。结合病例查阅相关文献,诊断IgG4-RD,给予甲强龙+环磷酰胺序贯治疗,第19天复查血清IgG4下降,第23个月复查腹部CT,腹膜后纤维化范围较入院时缩小。结论IgG4-RD是一类与血清中IgG4增高相关的慢性、系统性罕见病,可累及全身多个器官,是进行性纤维炎性反应性疾病,不容易被及时发现及诊治,而且激素治疗存在停药容易复发,激素加免疫抑制剂联合方案能达到更好的治疗效果。Objective The clinical characteristics of IgG4-related disease(IgG4-RD)involving both submandibular gland and peritoneum were reviewed,and its clinical manifestation and treatment was discussed to provide Rnonledge for primary care physicians.Methods By analyzing the clinical manifestations,laboratory tests,pathological examination,diagnosis,treatment,prognosis and literature review of a patient with IgG4-related disease admitted to the Department of Nephrology,the Third Affiliated Hospital of Zunyi Medical University on June 28,2019.Results A 62-year-old male patient was admitted to the hospital due to right double J tube placement for 1 month.Serum IgG4 was elevated,abdominal CT showed retroperitoneal fibrosis;Immunohistocehmistry of submandibular gland showed that scattered positive IgG(10-20 cells/HPF)with an IgG4/IgG positive cell ratio of approximately 0.7.The patient was diagnosed with IgG4-related disease and was given methylprednisolone and cyclophosphamide sequential therapy.Serum IgG4 decreased significantly on the 19th day,and abdominal CT examination on the 23rd month showed that the scope of retroperitoneal fibrosis was significantly reduced compared with that on admission.Conclusion IgG4-RD is a kind of chronic and systemic rare disease related to elevated serum IgG4,which can involve multiple organs throughout the body and is a progressive fibroinflammatory disease,and not easily to be detected and treated in time.The early treatment is glucocorticoid,but it is prone to relapse when discontinued,the combination of hormone+immunosuppressive regimen may be better to improve condition.

关 键 词：IGG4相关性疾病 免疫球蛋白G 浆细胞 甲强龙 环磷酰胺 

分 类 号：R593.2[医药卫生—内科学]