自身免疫性胰腺炎21例临床分析

The clinical analysis of autoimmune pancreatitis： a report of 21 cases

目的 总结自身免疫性胰腺炎（AIP）的临床特点及诊治体会.方法 收集2006年1月至2014年7月哈尔滨医科大学附属第一医院收治的21例AIP患者的临床资料,男性15例,女性6例,年龄36 -64岁.综合临床症状、影像学特点、血清学检查结果、诊断性治疗、组织病理学特点等探讨AIP的诊治.结果 AIP主要临床表现为不同程度梗阻性黄疸和上腹痛;血清IgG4升高16例（76.2％）,CA19-9升高6例（28.5％）,癌胚抗原升高3例（14.2％）;CT结果示胰腺弥漫性肿大9例,胰头局灶性肿大3例,胰腺局灶性占位9例.根据胰外病变表现、影像学、血清学及组织穿刺活检结果确诊11例（52.4％）,糖皮质激素诊断性治疗确诊3例（14.3％）,手术探查确诊7例（33.3％）.行胆总管空肠吻合术3例,胆囊空肠吻合术1例,胰十二指肠切除术2例,胰体尾联合脾切除术1例.病理检查显示胰腺导管周围纤维结缔组织增生,伴大量淋巴细胞和浆细胞浸润.除1例无症状的患者外,所有诊断明确的患者均接受正规的糖皮质激素治疗（口服泼尼松）后痊愈.随访时间3 - 93个月,4例（19.0％）复发,经大剂量糖皮质激素治疗后症状缓解.结论 AIP缺乏特异性的临床症状,早期诊断困难,误诊率高.临床医师应综合临床表现、影像学、血清学及组织病理学检查结果等进行确诊,以避免不必要的手术治疗.

Objective To investigate the clinical feature,diagnostic and therapeutic experience of autoimmune pancreatitis （AIP）.Methods Twenty-one patients with AIP treated in the First Affiliated Hospital,Harbin Medical University from January 2006 to July 2014 were analyzed retrospectively.There were 15 men and 6 women among the 21 cases and the age ranged from 36 to 64 years.The characters of diagnosis and treatment of AIP were explored through clinical symptoms,imaging features,serologic test results,diagnostic treatment,and histopathologic characteristics.Results All the patients showed obstructive jaundice and upper abdominal pain to different extents as major manifestations and the levels of serum IgG4,CA19-9,CEA were elevated in 16 cases （76.2%）,6 cases （28.5%） and 3 cases（14.2%）,respectively.CT showed diffuse enlargement of the pancreas in 9 cases,localized pancreatic head enlargement in 3 cases and focally pancreatic mass in 9 cases.AIP was confirmed by extrapancreatic involvement,radiological and serological results plus biopsy in 11 cases （52.4%）,interpretation of response to steroid in 3 cases （14.3%） and open laparotomy in 7 cases （33.3%）.Surgery included choledochojejunostomy in 3 cases,cholecystojejunostomy in 1 case,pancreaticoduodenectomy in 2 cases and distal pancreatectomy combined with splenectomy in 1 case.The pathologic results displayed massive lymphocytes and plasma cells infiltration in the pancreatic tissues as well as parenchymal fibrosis.Except for 1 patient who had no symptom,the regular steroid therapy was performed （oral prednisone） and all the patients were cured.The follow-up time range was from 3 to 93 months,4 cases（19.0%） were recurrent followed by the symptoms alleviated after the steroid was applied again.Conclusions AIP is rare and characterized by non-specific clinical manifestations so that the early diagnosis is difficult with a high misdiagnosis rate.The clinicians should strengthen the recognition of AIP and the definite diagnosis depends on the combination of clinical manifestations,radiological,serological and histopathological results so as to avoid the unnecessary operation.