44例IgG4相关性自身免疫性胰腺炎的临床特征分析

Clinical Features of IgG4-Related Autoimmune Pancreatitis: An Analysis of 44 Patients

摘要: 目的：探讨IgG4相关性自身免疫性胰腺炎的临床特征。方法：2014年9月至2020年6月青岛大学附属医院确诊并随访的44例IgG相关性自身免疫性胰腺炎的临床资料，分析其临床症状、胰腺外器官受累、实验室检测、影像学、治疗方法及预后。结果：男女比例为7.8:1 (39:5)，平均年龄(60 ± 12)岁，BMI中位数值为22.45 kg/m² (21.3425.30 kg/m²)。腹痛是(75%, 33/44)最为常见的首发症状，胰腺外器官受累包括胆管33例，涎腺6例，眼病6例，多发浅表和(或)深部淋巴结肿大4例，腹膜后纤维化1例。41例患者血清IgG4升高，36例高于正常值上限的2倍。影像学中胰腺弥漫性肿大32例，胰腺局灶性12例。5例患者行超声内镜引导下细针穿刺，未见明显淋巴浆细胞硬化性胰腺炎表现。与单纯应用激素治疗相比，减量期加用环磷酰胺复发率低。结论：IgG4相关性自身免疫性胰腺炎具有独特的临床、血清学指标、影像学和组织细胞病理学改变，激素联合免疫抑制剂或许有更好的治疗反应。

Abstract: Objective: To investigate the clinical features of IgG4-related autoimmune pancreatitis (IgG4-AIP). Methods: The clinical data of 44 cases of IgG4-AIP diagnosed and followed up in The Affiliated Hospital of Qingdao University from September 2014 to June 2020 were analyzed. The clinical symptoms, extra-pancreatic organ involvement, laboratory testing, imaging, treatment and prognosis were analyzed. Results: The male to female ratio was 7.8:1 (39:5), the mean age was (60 ± 12) years, and the median BMI was 22.45 kg/m² (21.34~25.30 kg/m²). Abdominal pain (75%, 33/44) was the most common initial symptom. Extra-pancreatic organ involvement included bile duct involvement in 33 patients, salivary gland involvement in 6 patients, eye disease in 6 patients, multiple superficial and/or deep lymph node enlargement in 4 patients, and retroperitoneal fibrosis in 1 patient. Serum IgG4 was elevated in 41 patients, and higher than 2 times the upper limit of normal in 36 patients. Imaging showed diffuse pancreatic enlargement in 32 patients and focal pancreas in 12 patients. Five patients underwent endoscopic ultrasonic-guided fine needle aspiration (EUS-FNA), and no obvious manifestations of lymphocytic sclerosing pancreatitis were observed. Compared with steroid therapy alone, cyclophosphamide had a lower recurrence rate during dose reduction. Conclusion: IgG4-associated autoimmune pancreatitis has unique clinical, serological, radiological and histopathological changes, and steroid combined with immunosuppressive agents may have a better therapeutic response.