自身免疫性胰腺炎19例临床总结与分析

A report of 19 cases of autoimmune pancreatitis: a clinical summary and analysis

目的总结自身免疫性胰腺炎（AIP）的临床特点并探讨其诊治经验。方法回顾性分析2011年1月至2015年12月收治的19例AIP患者的临床资料,总结AIP的诊断及治疗经验。结果 19例患者中,男女比例5.3︰1,中位发病年龄58岁（35-60岁）。常见症状为黄疸14例（73.7%）、腹痛14例（73.7%）。合并糖尿病7例（36.8%）,合并其它自身免疫病7例（36.8%）。检测CA199升高〉74 U/ml占12.5%,IgG4阳性率72.2%,抗核抗体阳性率66.7%。B超/CT/MRI/MRCP提示胰腺弥漫性肿大4例（21.1%）,局限性肿大14例（73.7%）,胰腺萎缩1例（5.3%）。14例（73.9%）行超声内镜（EUS）,其中6例行超声内镜引导下细针穿刺（EUS-FNA）。12例（63.2%）行内镜下逆行胰胆管成像（ERCP）并置入鼻胆引流管或胆管支架。误诊为胰腺癌3例（15.8%）,其中1例行手术治疗（5.3%）。12例（63.2%）使用激素治疗,其中1例因激素不耐受停药,1例因出现股骨头坏死停药,其余10例激素治疗均有效。结论 AIP是一种特殊类型的慢性胰腺炎,应通过临床症状并结合CA199、自身抗体、影像学检查、逐步普及IgG4检测、扩大EUS及EUS-FNA的使用等以提高AIP的正确诊断率。

Objective To summarize the clinical characteristics of autoimmune pancreatitis（ AIP） and to explore the experience in diagnosis and treatment of this disease. Methods The clinical data of 19 patients with AIP in this hospital during January 1,2011 to December 31,2015 were retrospectively analyzed. Results The ratio of males with females was 5. 3 ： 1 with an average age of 58（ 35 - 60） years old in these 19 patients. Their common symptoms were jaundice（ 73. 7%） and abdominal pain（ 73. 7%）. Seven cases（ 36. 8%） were complicated with diabetes and 7 cases（ 36. 8%） were associated with other autoimmune diseases. The percentage of patients with serum level of CA199 above 74 U / ml accounted for 12. 5%. The positive rates of serum level of immunoglobulin G4（ IgG 4） and antinuclear antibodies（ ANA） were 72. 2% and 66. 7%respectively. B ultrasound / CT / MRI / MRCP scannings showed diffuse enlargement of pancreas in 4 cases（ 21. 1%）,segmental enlargement of pancreas in 14 cases（ 73. 7%） and pancreatic atrophy in 1 case（ 5. 3%）. 14 patients（ 73. 9%） underwent endoscopic ultrasonography（ EUS）and 6 patients（ 31. 6%） underwent endoscopic ultrasonography guided fine needle aspiration（ EUS- FNA）. Endoscopic retrograde cholangio-pancreatography（ ERCP） had been done in 12 cases（ 63. 2%） with either endoscopic nasobiliary drainage（ ENBD） or biliary stent placement Three cases（ 15. 8%） were misdiagnosed as pancreatic cancer,and 1 case（ 5. 3%） had undergone surgical treatment. 12 cases（ 63. 2%） were treated with hormone,1 case discontinued the treatment due to intolerance,and 1 case discontinued the treatment due to necrosis of femoral head,and the remaining 10 cases were effective with hormone therapy. Conclusion AIP is a special type of chronic pancreatitis. We should have a overall consideration of clinical symptoms,serum levels of CA199 and autoantibodies,imaging examination and gradual universalization of IgG 4 testing,and expanding EUS and EUS- guided fine- needle aspiration in order to raise the rate of correct diagnosis of AIP.