自身免疫性胰腺炎的诊断与治疗

Diagnosis and treatment of autoimmune pancreatitis

目的探讨自身免疫性胰腺炎（AIP）的诊断与治疗。方法回顾性分析2009年1月至2013年12月天津医科大学肿瘤医院收治的25例AIP患者的临床资料。检测患者血清1球蛋白、IgG4，并行腹部影像学检查。诊断标准参照美国HISORt诊断标准或术后病理学检查结果。难以耐受手术的患者口服泼尼松治疗。术前影像学检查明确胰腺肿块，但无法明确肿块性质，不能完全排除恶性肿瘤，激素治疗无效，能够耐受手术的患者行手术治疗。采用门诊和电话方式进行随访，随访时间截至2014年12月。结果首发症状：黄疸16例，体质量明显减轻（体质量减轻〉10％标准体质量）4例，慢性腹泻（腹泻持续时间〉2个月或间歇期为2～4周的复发性腹泻）3例，腹痛2例。血清γ球蛋白异常13例，IgG4升高1例。影像学检查示胰腺肿块19例，胆管狭窄6例；有胰腺外器官受累11例。25例AIP患者中，10例行保守治疗；15例行手术治疗，其中13例行胰十二指肠切除术，2例行胰体尾联合脾切除术。10例保守治疗患者未出现不良反应。15例手术患者手术时间为（271±59）min，术中出血量为（268±109）mL，术后胃肠功能恢复时间为（3．8．±1．2）d10术后2例患者发生腹腔感染，其中1例发生胰瘘，1例发生胃排空障碍，予对症治疗后缓解。15例手术患者术后住院时间为（11．5±2．9）d10术后病理学检查：胰腺中心性腺泡萎缩，广泛性纤维化，伴淋巴浆细胞浸润，神经组织被淋巴浆细胞包绕，闭塞性静脉炎，IgG4阳性细胞绝对值〉50个／高倍视野，IgG4阳性细胞数〉40％IgG阳性细胞数。25例患者均获得随访，中位随访时间为27个月（6～47个月）。19例患者治疗6个月症状缓解，其中保守治疗患者7例，手术治疗患者12例，血清吖球蛋白和IgG4正常，胰腺肿块无复发。结论AIP临床表现为黄疸、血清叮球蛋白异常，影像学检查示胰腺肿块。外科手术治疗AIP安全、有效，但鉴于手术本身的创伤，应严格把握适应证。

Objective To investigate the diagnosis and treatment of autoimmune pancreatitis （AIP）. Methods The clinical data of 25 patients with AIP who were admitted to the Cancer Hospital of Tianjin Medical University between January 2009 and December 2013 were retrospectively analyzed. Patients received the test of serum γ-globulin and IgG4 and abdominal imaging examination. The revised HISORt or results of postoperative pathological examination were performed as diagnostic criteria. Patients who were unable to tolerate surgery were treated by oral prednisone. The focal masses were apparent in the pancreas by imaging examination, which cannot exclude the possibility of malignancy because of ambiguous pathologic characters of masses. Patients who received ineffective hormonal therapy and were able to tolerate surgery underwent surgery. All the patients were followed up by outpatient examination and telephone interview up to December 2014. Results Primary symptoms： jaundice was detected in 16 patients, obvious weight loss （weight loss 〉 10% standard body mass） in 4 patients, chronic diarrhea （ duration of diarrhea 〉 2 months or 2 weeks 〈 duration of intermittent diarrhea 〈 4 weeks） in 3 patients and abdominal pain in 2 patients. Abnormal level of serum γ-globulin and increasing level of IgG4 were detected in 13 and 1 pateints. The results of imaging examinations showed that pancreatic masses, stenosis of bile duct and extrapancreactic organ involvement were detected in 19, 6 and 11 patients. Of 25 patients with AIP, 10 underwent conservative treatment without adverse reaction and 15 underwent surgical treatment, including 13 of 15 patients undergoing pancreaticoduodenectomy and 2 of 15 patients undergoing resection of the body and tail of the pancreas ＋ spleneetomy. The operation time, volume of intraoperative blood loss and postoperative recovery time of gastrointes- tinal function in 15 patients undergoing surgery were （271± 59 ） minutes, （268 ± 109 ） mL and （ 3.8± 1.2） days.After operation, 2 patients were complicated with abdominal infection and had remission of symptoms by sympto- matic treatment, including 1 with pancreatic fistula and 1 with delayed gastric emptying. The duration of hospital stay of 15 patients undergoing surgery was （ 11.5 ± 2.9） days. The results of postoperative pathological examina- tion showed that there were central acinar atrophy, extensive fibrosis, lymphoplasmacytic cell infiltration, nerve tissue surrounded by the plasma cell lymphoma and obstructive phlebitis. The absolute value of positive cells of IgG4 was more than 50 high power field and number of positive cells of IgG4 was more than positive cells of 40% IgG. Twenty-five patients were followed up for a median time of 27 months （range, 6-47months）. Nineteen patients had remission of symptoms at month 6 after treatment with normal level of serum γ-globulin and IgG4 and without recurrence of pancreatic masses, including 7 receiving conservative treatment and 12 receiving surgical treatment. Conclusions The clinical signs of AIP are jaundice, abnormal serum -,/globulin and pancreatic masses which are found by imaging examination. Surgery is safe and effective for the treatment of AIP, while surgical indications should be strictly followed because of the surgical trauma.