免疫球蛋白G4相关性肾脏病合并EB病毒感染1例并文献复习

免疫球蛋白G4(immunoglobulin G4,IgG4)相关性疾病是一组纤维硬化性疾病,可由于多克隆IgG4阳性浆细胞浸润、组织纤维化导致多脏器功能衰竭[1].最常受累的脏器为胰腺、胆管、外分泌腺(涎腺、唾液腺等)、腹膜后和淋巴结[2],肾脏受累的比例大约为15％,当肾脏受累的时候被称为IgG4相关性肾脏病[1]...     

误诊为胆总管胰腺段癌的免疫球蛋白G4相关自身免疫性胰腺炎1例报告并文献复习

背景与目的：免疫球蛋白G4相关自身免疫性胰腺炎（IgG4-AIP）是一种较为罕见的胰腺炎,可表现为无痛性梗阻性黄疸或胰腺肿块等,与胰腺癌表现相似,术前影像学诊断较为困难,初诊时易误诊。笔者通过回顾1例IgG4-AIP患者的临床资料和诊断经过,并结合相关文献报道,对本病临床特点进行总结,以期为临床工作提供经验。方法：回顾性分析江苏省南通市第三人民医院2023年2月收治的1例误诊为胆总管胰腺段癌的IgG4-AIP患者的临床资料,结合国内外文献对该病的临床病理特点、诊疗过程及治疗方法进行分析总结。结果：患者,男性,78岁;因上腹部胀痛不适伴皮肤巩膜黄染1周入院。入院查体：皮肤、巩膜中度黄染,腹部无阳性体征。磁共振胰胆管造影示：胰头区增大、内见稍长T1稍长T2信号肿块,弥散加权像呈明显高信号,增强中度均匀强化,胰管及肝内外胆管扩张,胰头占位伴胰管及肝内外胆管扩张。通过讨论分析患者临床表现、实验室检查结果以及影像学资料等,考虑胰头部占位性病变合并梗阻性黄疸,恶性肿瘤不除外,认为有手术探查指征,遂行胰十二指肠切除术。术后病理检查确诊为IgG4相关硬化性疾病/IgG4相关硬化性胰腺炎。结论：IgG4...     

IgG4相关疾病累及多系统1例

<正>患者男,58岁,因"皮肤、巩膜黄染,饱餐后出现上腹部疼痛"就诊。实验室检查:丙氨酸基转移酶402 U/L,天冬氨酸氨基转移酶352 U/L,血清总胆红素119.6 μmol/L,直接胆红素85.1 μmol/L。腹部CT示胰腺弥漫性肿大,呈"腊肠状",周围可见低密度晕征,胆囊增大、壁增厚(图1A),符合胰腺炎、胆囊炎表现,并提示胆道低位梗阻。结合影像学表现,临床诊断为胰腺炎、胆汁淤积性黄疸、胆道下段梗阻。经抗感染、补液、经皮肝穿刺胆道引     

IgG4相关性甲状腺疾病

目的总结IgG4相关性甲状腺病与IgG4关系的研究进展。方法收集近年来国内外关于IgG4相关性甲状腺病的病理特征及其与IgG4关系的相关文献并作综述。结果IgG4相关性疾病是一类新近被认识的与IgG4淋巴细胞密切相关的慢性、系统性疾病,可单发,也可累及甲状腺及全身多个器官。在桥本甲状腺炎、里德尔甲状腺炎及甲状腺乳头状癌病灶中均发现了IgG4的表达。结论IgG4相关性甲状腺病是对甲状腺炎疾病的一种新的认识,对其的了解有助于对甲状腺炎患者提供合理的治疗手段。     

IgG4相关性疾病泌尿系统损害分析

目的 总结IgG4相关性疾病泌尿系统损害的临床特点,以其提高对该疾病的认识.方法 回顾性分析出现泌尿系统损害的IgG4相关性疾病患者6例的临床表现、实验室检查、影像学资料、病理表现、治疗及预后情况.结果 诊断为IgG4相关性疾病患者中6例存在泌尿系统损害,男女比例为4:2,中位年龄59岁(36～ 72岁),中位病程为10.5个月.除肾脏、输尿管受累外,所有患者均同时存在泌尿系统外的多器官受累.泌尿系统损害临床表现多样,包括肾功能异常、水肿和腹痛.所有患者均存在高球蛋白血症、血清IgG(中位值23.3 g/L)及IgG4亚型(中位值4227.0 mg/L)升高,肾小管源性蛋白尿;5例患者Scr明显升高(中位值237 μmol/L).影像学表现可分为4类:肾脏弥漫增大、CT多发低密度灶可伴不均匀强化灶、肾盂和(或)输尿管积水、肾脏萎缩.肾脏病理显示为弥漫纤维化伴肾间质大量淋巴细胞、浆细胞浸润的间质性肾炎表现,伴淋巴细胞、浆细胞IgG4免疫组化染色阳性.患者对中至大剂量糖皮质激素治疗反应良好,经治疗,临床症状改善,IgG、IgG4及Scr均明显降低.结论 IgG4相关性疾病泌尿系统损害临床表现多样化,多同时合并其他器官受累;肾组织病理学以IgG4阳性的淋巴细胞和浆细胞浸润的间质性肾炎为其突出特点;糖皮质激素治疗有效.     

充分认识IgG4相关自身免疫性胰腺炎诊治的特殊性

IgG4相关自身免疫性胰腺炎(IgG4-AIP)是由免疫介导的伴有纤维化的慢性炎症性疾病,患者可缺乏临床症状,或表现为腹痛、黄疸等非特异性消化道症状。IgG4分子是用于血清学和组织病理学诊断的一项重要标志物,但限于对该病发病机制了解的欠缺,IgG4也面临诊断特异性欠佳的窘境。自2011年正式命名以来,IgG4-AIP在诊断、治疗方面已取得广泛的共识,并形成系统且规范的诊治流程,但其复发率高,显著地影响患者的生活质量。加之IgG4-AIP与部分胰腺恶性肿瘤鉴别诊断困难,即使影像学领域不断发展精进,仍有少部分患者最终偏离最佳的治疗路径。IgG4-AIP的临床隐匿性导致我国的流行病学资料尚不完善,对于该病的诊治往往需要参考国际标准和指南。因此,及时总结国内的临床病例对今后的诊治有指导作用。     

IgG4相关性疾病累及肾盂疑诊肾盂癌1例报告

本文报道1例IgG4相关性疾病(IgG4-RD)累及肾盂病例。患者体检发现左肾盂占位, CTU、MRI、PET-CT检查均考虑左肾盂癌并多发淋巴结转移, 行CT定位左肾盂穿刺活检, 结合血清IgG4水平, 最终临床确诊IgG4-RD。口服甲泼尼龙治疗后肾盂病灶缩小, IgG4水平下降。IgG4-RD为一种新近认识的可累及全身多器官的纤维炎性疾病, 肾盂受累时易误诊为肾盂癌。结合多器官受累可能、血清学检查、影像学特征以及穿刺病理等结果, 能够获得正确诊断。本病对糖皮质激素治疗反应良好, 但易复发。     

IgG4相关性肾病的临床病理特征分析

目的探究本中心中国人群IgG4相关性肾病的发病情况、临床病理特征及预后。 方法回顾性分析2010年1月至2019年1月在东部战区总医院国家肾脏疾病临床医学研究中心行肾穿刺活检患者的临床病理资料,对肾组织中有大量浆细胞浸润的患者,重新测定肾活检时血清IgG4水平及肾组织IgG4阳性浆细胞浸润数目,结合肾外表现,筛选出确诊为IgG4相关性肾病的病例,并分析IgG4相关性肾病的临床特点、病理特征及肾脏预后。 结果44 784例肾活检患者中有22例确诊为IgG4相关性肾病。IgG4相关性肾病最常表现为蛋白尿(86.4%)及肾功能不全(81.8%),约68.2%患者存在肾外累及。高IgG血症、高IgG4血症、低补体血症的发生率分别为86.4%、84.2%、45.5%。肾脏组织学最常见的病理类型为IgG4相关间质性肾炎(90.9%),其次为膜性肾病(13.6%)。肾间质席纹状纤维化、鸟眼样改变分别占40.9%、54.5%。22例患者中,21例接受糖皮质激素治疗,2例失访,余20例中位随访时间为12个月,9例肾功能好转,10例肾功能平稳,1例肾功能减退并进展至终末期肾病。 结论IgG4相关性肾病发病率低,好发于中老年男性,最常表现为肾功能不全及蛋白尿。半数以上的患者有肾外表现。高IgG血症、高IgG4血症和低补体血症是其血清学特征。最常见的肾脏病理类型为间质性肾炎;大量IgG4阳性浆细胞浸润、肾间质席纹状纤维化或鸟眼样改变是其典型的病理特征。糖皮质激素仍是IgG4相关性肾病治疗首选的一线药物。     

国内IgG4相关性自身免疫性胰腺炎病例荟萃分析

目的 通过国内病例荟萃分析总结IgG4相关性自身免疫性胰腺炎（auto-immune pancreatitis,AIP）临床特点与诊治经验。方法 以“IgG4相关性自身免疫性胰腺炎”为主题关键词,选择中国知网数据库和万方数据库,检索2006年1月至2016年4月间公开发表的文献,并进行汇总分析。纳入标准符合我国2012年AIP诊断标准草案的IgG4相关性AIP病例共92例,分别记录其性别、年龄、首发症状、血清IgG4水平、影像学表现、病理学特征及治疗方法,总结该疾病的特征,为临床工作提供理论依据。结果 临床主要表现为黄疸49例（53.2%）,腹痛44例（47.8%）,体重减轻29例（31.5%）。血清学检查发现83例（90.2%）血清IgG4水平明显增高,20例（21.73%）抗核抗体阳性,31例（33.69%）类风湿因子阳性,5例（5.63%）中性粒细胞胞浆抗体弱阳性。肿瘤标志物CA 19-9升高35例（38.40%）,血清淀粉酶值升高11例（11.96%）。影像学表现,78例胰腺弥漫性肿大,14例局限性肿大。局限性肿大病例中,9例为胰头肿大,5例为胰腺体尾部肿大。8例出现胰腺假包膜结构,1例胰腺钙化。36例病理表现：胰腺纤维化,胰腺及其周围有大量淋巴细胞及浆细胞浸润,呈慢性炎症改变。3例免疫组化显示大量IgG阳性浆细胞浸润。92例中,17例因诊断不明或误诊实施手术治疗,82例使用糖皮质激素治疗,78例病情缓解,有效率达95.12%。结论 近年来随着IgG4相关性AIP的诊断标准的不断更新,该病的诊断率已有明显提高,但仍有误诊现象。临床医务人员仍需不断总结,不断分析,减少IgG4相关性AIP的误诊误治率。     

自身免疫性胰腺炎22例临床诊治分析

目的 探索自身免疫性胰腺炎（AIP）的临床特点及诊疗方法。方法 回顾性分析2017年9月至2020年7月在复旦大学附属中山医院胰腺外科诊治的22例AIP病人临床资料。通过记录分析AIP病人初诊及治疗阶段的临床表现,血清IgG4变化情况等辅助检查结果,激素治疗等情况,探索其诊治策略。结果 病人最初就诊时血清IgG4中位数为5.56（0.34～30.30）g/L,其中单纯胰腺受累者2.52（0.34～5.73）g/L,合并其他器官受累者7.59（0.66～30.30）g/L,合并胰腺外器官受累的AIP病人IgG4水平高于单纯胰腺受累的AIP病人（Z= 5.693,P=0.045）;22例病人中有9例接受激素治疗,治疗期间IgG4水平 3.7 （0.99,18.3）g/L,激素治疗2个月后IgG4水平中位数1.63（0.76,8.31）g/L,较未行激素治疗时明显下降（Z=-2.093,P=0.038）。8例病人（激素治疗5例,未激素治疗3例）复发时,IgG4较前均有不同程度升高（8/8,100%）,中位升高数值4.14（0.61,14.81）g/L;4例停药病人中,2例病人IgG4水平未恢复到正常（2/4,50%）。另有13例病人未接受激素治疗,治疗随访时10例（10/13,76.9%）病人处于临床缓解期。结论 在AIP病人中,血清IgG4在多器官受累时升高更为显著。使用激素治疗的AIP病人,开始激素治疗2个月后,复测IgG4较治疗前显著降低。部分轻症的AIP病人,经对症治疗后,可自行缓解。     

Surgical treatment for a patient with immunoglobulin G4-related lung disease

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune fibroinflammatory disease, and isolated lung disease is a rare phenomenon. IgG4-RD frequently mimics primary pulmonary malignancy. It has been described in association with malignancies, including lung cancer, but it remains unclear if it increases the risk of malignancy. We report the case of a patient who had a left lower lobectomy after relapse while receiving corticotherapy. The pathological findings confirmed the diagnosis of IgG4-RD. After 2 months, the thoracic computerized tomography scan showed an important left pleural effusion that could be a consequence of the recent intervention or a relapse of IgG4-RD.     

Early pancreatic cancer in Ig G4-related pancreatic mass: A case report

BACKGROUND Ig G4-related disease can manifest diversely, including autoimmune pancreatitis and Ig G4-related cholangiopathy. We are reporting a very unusual cause of pancreatic cancer triggered in a previously unknown Ig G4-related disease.CASE SUMMARY A 75-year-old man was diagnosed with a 43 mm × 33 mm pancreatic head tumor after consulting for abdominal pain and jaundice. A pancreaticoduodenectomy was carried out uneventfully, and the histopathology report showed an early stage of acinar-cell pancreatic cancer. The patient reconsulted on the 30 th postoperative day with fever, jaundice and asthenia. Magnetic resonance cholangiopancreatography evidenced an extense bile duct stricture. A percutaneous biliary drainage proved to be ineffective, even after exchanging it with larger bore drainage. Reviewing the surgical specimen, features compatible with Ig G4-related disease were observed. Consequently, empiric treatment with steroids was initiated achieving excellent results.CONCLUSION Ig G4-related disease may cause chronic inflammation of the pancreas and can condition pancreatic malignancies.     

IgG4-related disease of the ileocecal region mimicking malignancy: A case report

INTRODUCTION

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease characterized by chronic fibrosing inflammation with abundant IgG4-positive plasma cells, and responds well to steroids. Previous reports of IgG4-RD have focused on pancreatic and extrapancreatic including the gastrointestinal tract, however, the colonic IgG4-RD is rare.

PRESENTATION OF CASE

We herein report the case of a 74-year-old female with edematous wall thickening of the terminal ileum to the lower ascending colon confirmed by several preoperative imaging studies, who underwent right hemi-colectomy for suspected malignant lymphoma. The resected specimen showed an irregular wall thickness with subserosal sclerosis, and the lesion was 10 cm in length from the terminal ileum to the ascending colon. The patient was diagnosed with IgG4-RD by pathological examinations, which demonstrated an increased number of IgG4-positive plasma cells (150/HPF), and an elevated IgG4/IgG ratio (50%).

DISCUSSION

Gastrointestinal IgG4-RD appears to be difficult to diagnose prior to surgical resection because of its rarity, and the similarity of its features to malignancy.The measurement of the serum IgG4 levels, immunohistochemical examination of biopsy specimens and use of several imaging modalities might help us to diagnose the disease without surgical resection, and this disease can generally be treated with steroid therapy. However, surgical resection for IgG4-RD may still be also necessary for patients with concerns regarding malignancy or with intractable gastrointestinal obstruction caused by this disease.

CONCLUSION

Gastrointestinal IgG4-RD often mimics malignancy, and we should therefore consider this disease in the differential diagnosis of colonic lesions in order to optimize the treatment.     

Immunoglobulin G4-related inflammatory pseudotumor of the lung

An 82-year-old man presented with a nodule in the right S²a of the lung as seen by chest computed tomography (CT). He had undergone treatment for chronic obstructive lung disease. He had a 53-year history of smoking 20 cigarettes a day. Subsequent to the appearance of the nodule in the right S²a, the CT images revealed consolidations in the right S²b, right S³, and left S⁵. The nodule in the right S²a was diagnosed as squamous cell carcinoma after performing video-assisted thoracoscopic wedge resection of the lung. After 4 months, the size of the consolidation in the right S²b increased. Recurrence of lung cancer was suspected. Using transbronchial lung biopsy, the consolidation in the left S⁵ was diagnosed as organizing pneumonia; therefore, right upper lobectomy was performed. The consolidations in the right S²b and right S³ were diagnosed as inflammatory pseudotumors with infiltrations of immunoglobulin G4-positive plasma cells.     

Immunoglobulin G4-related inflammatory pseudotumor of the lung

A 61-year-old man was pointed out a solitary nodule located in the left lung (S9) measuring 18 × 29 mm in size along with lymphadenopathy by chest computed tomography (CT). Positron emission tomography( PET) scan showed a positive sign corresponding to the nodule[ standardized uptake value (SUV) max 5.8]. No diagnostic material was obtained from the transbronchial tumor biopsy, since it was difficult to rule out malignancy, surgical biopsy was performed with sampling of mediastinal lymph nodes. Histopathological examination showed marked infiltration of inflammatory cells, many of which were demonstrated to be immunoglobulin (Ig) G4-positive plasma cells by immunohistochemical staining. Hence, IgG4-related inflammatory pseudotumor of the lung was diagnosed.     

IgG4相关硬化性胆管炎一例并国内文献复习

目的探讨我国Ig G4相关硬化性胆管炎(Ig G4-RSC)的临床特点、治疗策略以及预后。方法收集国内资料完整的符合Ig G4-RSC诊断标准的36例病人结合我院诊治的一例进行研究分析,探讨该病的临床特点、诊断、治疗以及预后。结果男28例,女9例。好发年龄于50-60岁之间。常见的临床表现主要为梗阻性黄疸(89%)、上腹部不适或疼痛(49%)和消瘦(27%)。15例接受血清学Ig G4检查有14例(93%)升高。22例Ig G检查中21例(95%)升高,29例CA19-9检查中18例(62%)升高。影像学检查:37例中33例胆总管下段狭窄(89%),4例肝门区胆管和胆总管中下段狭窄(11%)。37例均合并自身免疫性胰腺炎(AIP),多发淋巴结肿大3例(7%)。14例接受外科手术,术后病理证实Ig G4-RSC,5例术后加用激素治疗。23例行激素治疗,其中6例施行胆管支架植入,症状均有缓解。6例复发,再次行激素治疗后症状缓解。结论 Ig G4-RSC好发于中老年男性,常以梗阻性黄疸就诊,血清学检查Ig G4多有升高,常合并有自身免疫性胰腺炎,病理学检查有助于诊断(术前较难获得),激素治疗有效,复发再次激素治疗,症状仍能缓解。