自身免疫性胰腺炎的诊治及外科手术价值研究

Diagnosis and treatment of autoimmune pancreatitis and the value of surgery

目的总结自身免疫性胰腺炎(AIP)的诊治经验,探讨外科手术在其治疗中的价值。方法回顾性分析2013年1月至2017年6月福建医科大学附属协和医院收治的17例AIP病人的临床资料。观察临床表现、血液检查结果、影像学表现、诊断及治疗、随访情况。结果 (1)临床表现:17例病人中,7例有梗阻性黄疸,8例上腹痛,1例反复腹泻,1例为因体检发现胰腺肿物。共有11例病人存在胰腺外器官受累。(2)血液检查:13例病人血清免疫球蛋白G4(IgG4)升高,2例正常,2例未查。4例病人CA19-9升高,13例正常。3例病人血清淀粉酶轻度升高,14例正常。(3)影像学检查:所有17例病人均行胰腺CT平扫+增强检查,12例CT表现为弥漫型,5例CT表现为局灶型。(4)诊断及治疗:17例病人均确诊为1型AIP。17例病人中有3例因影像学疑似胰腺癌行手术治疗,术后病理学检查证实为AIP,包括胰十二指肠切除术2例和胰体尾+脾切除术1例。14例AIP病人接受初始口服激素治疗,11例缓解,1例因CA19-9持续升高行胰体尾+脾切除术,1例因胰头假性囊肿逐渐增大伴梗阻性黄疸行胰十二指肠切除术,1例因胰体假性囊肿伴腹痛行胰体囊肿空肠内引流术。(5)随访情况:17例病人均获得随访,平均随访时间为29(12～66)个月。3例初始接受手术的病人术后疾病缓解,复查未见复发。14例接受激素初始治疗的病人,11例有效,疾病缓解;另3例接受手术后继续口服激素治疗3个月,未见复发。结论 AIP的诊断须综合临床表现、血清学、影像学和病理学等检查结果,应用激素是治疗AIP最主要的方法,但外科手术仍占有重要地位,尤其适用于难以与胰腺癌鉴别的局灶型AIP和激素治疗效果欠佳的AIP合并假性囊肿。

Objective To summarize the experience of diagnosis and treatment of autoimmune pancreatitis(AIP)and to investigate the value of surgery in the management of AIP.Methods The clinical data of 17 patients with AIP who were admitted to Fujian Medical University Union Hospital between January 2013 and June 2017 were analyzed retrospectively.Observed indexes included clinical manifestations,blood test results,imaging findings,diagnosis and treatment and follow-up status.Results(1)Clinical manifestations:of the 17 patients,7 demonstrated obstructive jaundice,8 had upper abdominal pain,1 had recurrent diarrhea and 1 was found a pancreatic tumor by health checkup.Extrapancreatic organ involvement was detected in 11 of the 17 patients.(2)Blood test results:13 patients underwent serum IgG4 detection and increased expression of IgG4 was found in 13 patients.Increased expression of serum CA199 was detected in 4 patients.Elevated level of serum amylase was found in 3 patients.(3)Imaging findings:all the 17 patients underwent enhanced computed tomography(CT)scan of pancreas.Diffused enlargement of pancreas was observed in 12 patients and localized pancreatic enlargement was observed in 5 patients.(4)Diagnosis and treatment:All of the 17 patients were diagnosed as type 1 AIP.Three of the 17 patients underwent operation due to suspicion of pancreatic cancer and were diagnosed as AIP based on postoperative pathological examinations,including 2 pancreaticoduodenctomy and 1 distal pancreatectomy with splenectomy.Fourteen patients received initial steroid treatment.After steroid treatment,11 patients achieved remission,1 underwent distal pancreatectomy with splenectomy due to continuous elevation of serum CA199,1 underwent pancreaticoduodenctomy because of a continuous enlarged pancreatic pseudocyst in the head of pancreas with obstructive jaundice,and 1 underwent cystjejunostomy due to a pancreatic pseudocyst in the body of pancreas with abdominal pain.(5)Follow-up status:All of the 17 patients were followed up for an average time of 29 months(range,12-66 months).Three patients who initially underwent pancreatectomy achieved remission after operation and no recurrence was observed.Of the 14 patients who received initial steroid treatment,11 got remission and the other three patients received maintenance steroid treatment for 3 months after operation and no recurrence was observed.Conclusion The diagnosis of AIP should be based on the clinical manifestations,blood test results,imaging findings and pathological examinations.Steroid is the most critical therapy for AIP,but surgery is still important for the management of AIP,especially for localized AIP which is difficultly distinguished from pancreatic cancer and AIP with pancreatic pseudocyst which is refractory to steroid treatment.