IgG4相关性自身免疫性胰腺炎临床特点及诊断策略评价

Clinical characteristics of IgG4-related autoimmune pancreatitis and its diagnostic strategy evaluation

目的探讨IgG4相关性自身免疫性胰腺炎(IgG4-AIP)临床特点及诊断。方法回顾性分析2013年1月至2018年12月在中山大学附属孙逸仙纪念医院诊治的24例IgG4-AIP患者临床资料。患者均签署知情同意书,符合医学伦理学规定。其中男20例,女4例;年龄46~84岁,中位年龄61岁。根据IgG4相关性疾病(IgG4-RD)综合诊断标准和国际共识诊断标准(ICDC)诊断IgG4-AIP患者。分析其临床表现,实验室、影像学、病理学检查情况,研究其诊断策略。结果临床表现腹痛12例,黄疸10例,腹胀6例,消瘦3例,无症状2例。单纯胰腺病变7例,其余合并1~5个器官损害,其中累及胆道系统10例,淋巴结8例,唾液腺和肺各3例,胃肠和血管各2例,肝、眼、腹膜后各1例。血清IgG4中位数为18(2~117)g/L。影像学检查胰腺局限肿块13例,弥漫性肿大11例,胰管狭窄9例。胰腺病理学检查示纤维化4例,IgG4+细胞>10个/高倍镜视野,且IgG4^+/IgG^+>40%12例。根据IgG4-RD综合诊断标准诊断10例,根据ICDC诊断14例。误诊胰头癌、胆管癌8例,行胰腺切除或活检术,术后病理证实为IgG4-AIP。结论IgG4-AIP临床表现无特异性,局限肿块病变易被误诊,可综合运用血清IgG4检测、影像学、病理学、ICDC标准,提高诊断率。

Objective To investigate the clinical characteristics and diagnosis of IgG4-related autoimmune pancreatitis(IgG4-AIP).Methods Clinical data of 24 IgG4-AIP patients admitted to Sun Yat-sen Memorial Hospital Affiliated to Sun Yat-sen University from January 2013 to December 2018 were retrospectively analyzed.The informed consents of all patients were obtained and the local ethical committee approval was received.Among them,20 patients were male and 4 female,aged 46-84 years with a median age of 61 years.Patients with IgG4-AIP were diagnosed according to the comprehensive diagnostic criteria of IgG4-related disease(IgG4-RD)and the International Consensus Diagnostic Criteria(ICDC).Its clinical manifestations,laboratory,imaging,pathological examinations and the diagnostic strategy were analyzed.Results Clinical manifestations included abdominal pain in 12 cases,jaundice in 10,abdominal distension in 6,emaciation in 3 and no symptoms in 2,respectively.7 patients were diagnosed with simple pancreatic lesions and the remaining cases were complicated with 1-5 organs involvement,which were biliary tract involvement in 10 cases,lymph nodes in 8 cases,salivary glands in 3 cases,lungs in 3 cases,gastrointestinal tract in 2 cases, blood vessels in 2 cases, and liver, eye, retroperitoncum in 1 case, respectively. The medianlevel of serum IgG4 was 18(2-117) g/L. Imaging examination indicated localized pancreatic masses in 13 cases,diffuse swelling in 11 cases and pancreatic duct stenosis in 9 cases, respectively. Pathological examinationof the pancreas demonstrated fibrosis in 4 cases, IgG4+ cell count>10/high power field, and IgG4^+/IgG^+>40% in 12 cases. According to comprehensive diagnostic criteria of IgG4-RD, 10 cases were diagnosed withIgG4-AIP and 14 cases were diagnosed with IgG4-AIP according to ICDC. 8 cases were misdiagnosedas pancreatic head cancer or cholangiocarcinoma. Pancreatic resection or biopsy was performed and thepostoperative pathological examination confirmed the diagnosis of IgG4-AIP. Conclusions Clinicalmanifestations of IgG4-AIP are nontypical. The localized lesions are easy to be misdiagnosed. The diagnosticrate can be improved by comprehensive application of serum IgG4 detection, imaging and pathologicalexaminations, and ICDC criteria.