43例IgG4相关性疾病患者临床诊治分析

  目的  分析总结IgG4相关性疾病（IgG4-related disease, IgG4-RD）患者临床、病理特征及诊治经验。  方法  回顾性分析2013年1月?2017年12月四川大学华西医院收治的43例IgG4-RD患者的临床资料（包括临床表现、实验室检查、影像学及病理学检查，诊断、误诊及治疗情况）。  结果  43例IgG4-RD患者中，男女性别比为3∶1，年龄（51.3±15.9）岁，临床表现为腹痛、黄疸、乏力等消化道症状者11例（IgG4相关性胆管炎5例，IgG4相关性自身免疫性胰腺炎6例），占25.6%；眶周受累患者10例，占23.3%，淋巴结肿大患者9例，占20.9%，发热患者6例，占14.0%；42例患者行免疫自身抗体检查，抗核抗体（ANA）升高14例；40例患者行免疫球蛋白检查，IgG升高25例，IgA升高12例，IgE升高29例；6例患者行直接抗人球蛋白实验，4例为阳性；血清免疫球蛋白G4亚型检测显示，35例患者高于1 350 mg/L；影像学检查显示，眶周受累患者CT特征为眼眶内软组织结节；胆道及胰腺受累患者腹部增强CT扫描特征为肝内胆管扩张、胆总管扩张，管壁增厚强化，管腔变窄，胰腺受累显示胰腺肿大或软组织密度影；组织病理学检查免疫组化染色表现为IgG4阳性细胞（3～>500个/高倍视野）浸润，淋巴细胞CD20、CD3ε或浆细胞CD138阳性。43例患者中5例因误诊行手术治疗。所有患者确诊后采用激素及免疫抑制剂等药物治疗，临床症状均好转。  结论  IgG4-RD临床表现多样，累及多器官，临床上应加强对IgG4-RD的规范诊断和治疗。

Diagnosis and Treatment of 43 Patients with IgG4-related Disease

  Objective   To summarize the clinicopathological characteristics, diagnosis and treatment of IgG4-related disease (IgG4-RD).  Methods  The clinical data of 43 cases with IgG4-RD diagnosed from January 2013 to December 2017 were retrospectively analyzed. The clinical data of the patients including clinical characteristics, accessory examinations, diagnosis, and treatment were collected.  Results  Among the 43 patients with IgG4-RD, the ratio of male to female was 3∶1, the mean age was (51.3±15.9) years. Eleven patients had gastrointestinal symptoms, including 5 cases of IgG4-related cholangitis with the feature of dilation of the biliary system and narrowing of the lumen in the abdominal enhanced CT scans, and 6 cases of IgG4-related autoimmune pancreatitis with the feature of pancreatic enlargement or soft tissue density shadow in the abdominal enhanced CT scans. There were 10 cases (23.3%) with periorbital involvement, with the feature of intraorbital soft tissue nodule in the CT scan. Besides, 9 cases (20.9%) had lymphadenopathy, 6 cases (14.0%) had fever. The results of autoimmune antibody tests showed that 14 of 42 patients had increased antinuclear antibody (ANA). Among 40 patients who underwent immunoglobulin tests, 25 cases had elevated IgG, 12 cases had increased IgA, and 29 cases had increased IgE. Coombs test were performed for 6 cases and 4 patients were positive. Serum immunoglobulin G4 subtypes showed that the IgG4 levels of 35 patients were higher than 1 350 mg/L. The immunohistochemistry showed that IgG4 (+) cells (3->500/HPF) were infiltrated, with the CD20 (+), CD3ε (+) or CD138 (+). Among the 43 patients, 5 patients underwent operations due to misdiagnosis. All patients were treated with steroid and immunosuppressive agents after diagnosis, and their clinical symptoms were improved.   Conclusion  The clinical symptoms of IgG4-RD are various, involving multiple organs. Therefore, the standardized diagnosis and treatment of IgG4-RD should be strengthened.