CAJ | 학술논문

目的：探讨IgG4相关自身免疫性胰腺炎（AIP）的临床特点。方法回顾性研究2003年1月至2012年12月在中山大学孙逸仙纪念医院肝胆胰外科接受诊治并经病理学确诊的12例IgG4相关AIP患者临床资料。所有患者均签署知情同意书，符合医学伦理学规定。患者均为男性，平均年龄为（54±13）岁，均被误诊为胰头癌行胰头十二指肠切除术。收集患者临床表现及实验室、影像学、病理学检查等资料。患者术后接受随访，观察治疗情况及疗效。结果12例患者中腹痛7例、黄疸7例、消瘦6例、无明显症状1例，合并糖尿病3例、慢性下颌下腺炎1例。患者血、尿淀粉酶均正常，血GGT升高12例、糖链抗原19-9（CA19-9）轻度升高9例、癌抗原（CA）125升高3例、癌胚抗原（CEA）升高2例。增强CT示胰头局部肿大11例，伴胰管轻度扩张3例。MRI示胰体呈“腊肠样”改变4例，胰周呈“包鞘样”改变5例。磁共振胰胆管成像（MRCP）示胰头段胰管及胆总管下段狭窄，其中4例远端胰管轻度扩张。影像学检查未发现局部侵犯。病理学检查表现为淋巴浆细胞硬化性胰腺炎，免疫组织化学方法（免疫组化法）染色IgG4阳性。12例术后患者间断出现腹痛，其中7例需服用止痛药。1例患者经泼尼松治疗后症状缓解。结论 IgG4相关AIP临床表现与胰腺癌相似，易误诊为胰腺癌。其主要临床特点为血CA19-9轻度升高；影像学检查见胰头局部肿大、胰体呈“腊肠样”、胰周呈“包鞘样”改变，且无局部侵犯；病理学检查为淋巴浆细胞硬化性胰腺炎，免疫组化法染色IgG4阳性；肾上腺皮质激素治疗有效。
목적：탐토IgG4상관자신면역성이선염（AIP）적림상특점。방법회고성연구2003년1월지2012년12월재중산대학손일선기념의원간담이외과접수진치병경병이학학진적12례IgG4상관AIP환자림상자료。소유환자균첨서지정동의서，부합의학윤리학규정。환자균위남성，평균년령위（54±13）세，균피오진위이두암행이두십이지장절제술。수집환자림상표현급실험실、영상학、병이학검사등자료。환자술후접수수방，관찰치료정황급료효。결과12례환자중복통7례、황달7례、소수6례、무명현증상1례，합병당뇨병3례、만성하합하선염1례。환자혈、뇨정분매균정상，혈GGT승고12례、당련항원19-9（CA19-9）경도승고9례、암항원（CA）125승고3례、암배항원（CEA）승고2례。증강CT시이두국부종대11례，반이관경도확장3례。MRI시이체정“석장양”개변4례，이주정“포초양”개변5례。자공진이담관성상（MRCP）시이두단이관급담총관하단협착，기중4례원단이관경도확장。영상학검사미발현국부침범。병이학검사표현위림파장세포경화성이선염，면역조직화학방법（면역조화법）염색IgG4양성。12례술후환자간단출현복통，기중7례수복용지통약。1례환자경발니송치료후증상완해。결론 IgG4상관AIP림상표현여이선암상사，역오진위이선암。기주요림상특점위혈CA19-9경도승고；영상학검사견이두국부종대、이체정“석장양”、이주정“포초양”개변，차무국부침범；병이학검사위림파장세포경화성이선염，면역조화법염색IgG4양성；신상선피질격소치료유효。
Objective To investigate the clinical features of IgG4 related autoimmune pancreatitis (AIP). Methods Clinical data of 12 patients pathologically diagnosed as IgG4 related AIP in Department of Hepatopancreatobiliary Surgery, Sun Yat-sen Memorial Hospital of Sun Yat-sen University from January 2003 to December 2012 were retrospectively analyzed. The informed consents of all patients were obtained and the ethical committee approval was received. All the patients were males with a mean age of (54±13) years old. All were misdiagnosed as pancreatic head carcinoma and received pancreatoduodenectomy. Data of the clinical manifestations, laboratory, imaging and pathological examinations were collected. The patients were followed up after operations, the treatment and outcome were studied. Results The clinical manifestations of the 12 patients were abdominal pain (n=7), jaundice(n=7), emaciation(n=6) and no obvious symptom (n=1). Three cases were combined with diabetes, 1 case with chronic sialadenitis of submandibular gland. The patient's blood and urine amylase were normal. Blood gamma-glutamyl transpeptidase (GGT) increase was observed in 12 cases. Mild increase of carbohydrate antigen 19-9 (CA19-9) was observed in 9 cases. Cancer antigen (CA) 125 increase was observed in 3 cases, and carcinoembryonic antigen (CEA) increase in 2 cases. Through contrast-enhanced CT, partial pancreatic head enlargement was observed in 11 cases, in which 3 cases were observed combining with mild expansion of pancreatic duct. Through magnetic resonance imaging (MRI), sausage-like change of pancreas body was observed in 4 cases, peripancreatic sheath-like change in 5 cases. Through magnetic resonance cholangiopancreatography (MRCP), stenoses in the pancreatic duct of pancreatic head segment and distal common bile duct were observed including 4 cases of mild expansion in the distal pancreatic duct. Local invasion was not observed by imaging examinations. Lymphoplasmacytic sclerosing pancreatitis was determined by pathological examination. Positive expression of IgG4 was observed by immunohistochemistry. Twelve cases suffered from discontinuous abdominal pain after operations, in which 7 cases needed acesodyne. One case relieved after using prednisone. Conclusions Clinical manifestations of IgG4 related AIP are similar to pancreatic carcinoma and can be misdiagnosed as pancreatic carcinoma easily. The main clinical features are mild increase of blood CA19-9. Partial pancreatic head enlargement, pancreas body sausage-like change, peripancreatic sheath-like change, and no local invasion are observed by imaging examinations. Lymphoplasmacytic sclerosing pancreatitis is determined by pathological examination. Positive expression of IgG4 is observed by immunohistochemistry. Adrenocortical hormone treatment is effective.