自身免疫性胰腺炎误诊误治17例临床分析

Objective To summarize the reasons of mis-diagnosis and mis-treatment of autoimmune pancreatitis (AIP). Methods Clinical data of 17 patients with AIP,who were admitted to the hospital from May 2005 to July 2010 and experienced mis-diagnosis and mis-treatment, were retrospectively analyzed. Results The main clinical manifestations included epigastric pain (13 cases),progressive obstructive jaundice (12 cases), fever (6 cases) and weight loss (9 cases). Fifteen patients had extrapancreatic organ involvemnet, including allergic rhinitis, swelling of lymphoglandulae submaxillares, swelling of submaxillary gland, allergic asthma, rheumatoid arthritis, Sjogren syndrome, diabetes mellitus, primary sclerosing cholangitis and autoimmune hepatitis. Of these 17 cases, 11 cases presented with high serum globulin, 14 cases with high serum IgG, 13 cases with high serum γ-globulin, 13 cases with positive anti-nuclear antibody and 2 cases with positive anti-insulin IgG antibody. The abdominal imaging demonstrated that 15 patients had diffuse enlargement of the pancreas with diffuse or segmental narrowing of main pancreatic duct, narrowing of the intrapancreatic common bile duct, dilation of the proximal biliary duct and gallbladder enlargement. Focal enlargement of the pancreas was found in 2 cases. Thirteen cases were misdiagnosed as pancreatic carcinoma. Among them, 4 cases underwent pancreaticoduodenectomy and 7 cases underwent choledochojejunostomy. Two cases were misdiagnosed as end stage of cancer that lost therapeutic chance. Another 4 cases were misdiagnosed as chronic pancreatitis. Steroid therapy was administered in all patients with satisfactory response. All patients were followed-up for 15 months (ranged from 6 months to 45 months), and recurrence was found in 4 cases. Satisfactory response was found in patients treated with steroid for the second time. No pancreatic cancer was found in these patients in the follow up period. Conclusion The main causes of mis-diagnosis and mis-treatment of AIP may be contributed by difficulty in differentiating AIP from pancreatic carcinoma based on clinical manifestations and inadequate knowledge of AIP as well as insufficient attention to AIP in China.     

自身免疫性胰腺炎误诊误治17例临床分析

目的 总结自身免疫性胰腺炎(AIP)误诊误治的原因.方法 回顾性分析2005年5月至2010年7月间我院收治的17例有误诊误治经历的AIP患者的临床资料.结果 AIP的主要临床症状包括腹部轻度疼痛13例、进行性黄疸12例、发热6例、体重减轻9例.15例伴随一种或一种以上胰腺外脏器的自身免疫性疾病,包括过敏性鼻炎、颌下淋巴结肿大、颌下腺肿大、过敏性哮喘、类风湿性关节炎、干燥综合征、糖尿病、原发性硬化性胆管炎和自身免疫性肝炎.实验室检查方面,血清球蛋白增高11例、IgG增高14例、γ球蛋白增高13例、抗核抗体阳性13例、抗胰岛素IgG抗体阳性2例.影像学检查方面,胰腺弥漫性肿大15例,伴主胰管弥漫性狭窄或节段性狭窄、胰腺段胆总管狭窄、近端胆管扩张、胆囊增大;胰头、钩突局灶性肿大2例.17例中有13例误诊为胰腺癌,其中4例行胰十二指肠切除术,7例行胆肠吻合术,2例被认为肿瘤晚期失去治疗机会;4例被误诊为普通慢性胰腺炎.确诊AIP后,17例患者均接受糖皮质激素治疗,效果显著.随访6～45个月(平均15个月),4例复发,再次子糖皮质激素治疗效果好.随访期间未发现胰腺癌.结论 AIP 临床特点与胰腺癌相似,加之我国现阶段对AIP认识不足、重视不够,是导致误诊和误治的主要原因.

Abstract：

Objective To summarize the reasons of mis-diagnosis and mis-treatment of autoimmune pancreatitis (AIP). Methods Clinical data of 17 patients with AIP,who were admitted to the hospital from May 2005 to July 2010 and experienced mis-diagnosis and mis-treatment, were retrospectively analyzed. Results The main clinical manifestations included epigastric pain (13 cases),progressive obstructive jaundice (12 cases), fever (6 cases) and weight loss (9 cases). Fifteen patients had extrapancreatic organ involvemnet, including allergic rhinitis, swelling of lymphoglandulae submaxillares, swelling of submaxillary gland, allergic asthma, rheumatoid arthritis, Sjogren syndrome, diabetes mellitus, primary sclerosing cholangitis and autoimmune hepatitis. Of these 17 cases, 11 cases presented with high serum globulin, 14 cases with high serum IgG, 13 cases with high serum γ-globulin, 13 cases with positive anti-nuclear antibody and 2 cases with positive anti-insulin IgG antibody. The abdominal imaging demonstrated that 15 patients had diffuse enlargement of the pancreas with diffuse or segmental narrowing of main pancreatic duct, narrowing of the intrapancreatic common bile duct, dilation of the proximal biliary duct and gallbladder enlargement. Focal enlargement of the pancreas was found in 2 cases. Thirteen cases were misdiagnosed as pancreatic carcinoma. Among them, 4 cases underwent pancreaticoduodenectomy and 7 cases underwent choledochojejunostomy. Two cases were misdiagnosed as end stage of cancer that lost therapeutic chance. Another 4 cases were misdiagnosed as chronic pancreatitis. Steroid therapy was administered in all patients with satisfactory response. All patients were followed-up for 15 months (ranged from 6 months to 45 months), and recurrence was found in 4 cases. Satisfactory response was found in patients treated with steroid for the second time. No pancreatic cancer was found in these patients in the follow up period. Conclusion The main causes of mis-diagnosis and mis-treatment of AIP may be contributed by difficulty in differentiating AIP from pancreatic carcinoma based on clinical manifestations and inadequate knowledge of AIP as well as insufficient attention to AIP in China.     

MRCP and MRI findings in 9 patients with autoimmune pancreatitis

AIM: To evaluate magnetic resonance cholangiopancre-atography (MRCP) findings in conjunction with magnetic resonance (MR) images in autoimmune pancreatitis (AIP) patients. METHODS: Nine patients with AIP underwent MRI, MRCP, endoscopic retrograde cholangiopancreatography (ERCP), computed tomography, and ultrasonography. The MRCP and MR images taken before and after steroid therapy were reviewed and compared with other imaging modalities. The MRCP findings of the AIP cases were compared to those of 10 cases with carcinoma of the head of the pancreas. RESULTS: On MRCP, the narrowed portion of the main pancreatic duct noted on ERCP was not visualized, while the non-involved segments of the main pancreatic duct were visualized. The degree of upstream dilatation of the proximal main pancreatic duct was milder than that seen in cases of pancreatic carcinoma. Stenosis or obstruction of the lower bile duct was detected in 8 patients. MR images showed enlargement of the pancreas with decreased signal intensity on T1-weighted MR images, increased signal intensity on T2-weighted MR images, and, in 3 patients, a hypointense capsule-like rim. After steroid therapy, the previously not visualized portion of the main pancreatic duct was seen, along with improvement of the bile duct stenosis. Pancreatic enlargement decreased, and the abnormal signal intensity on both T1- and T2-weighted MR images became isointense. CONCLUSION: MRCP cannot differentiate irregular narrowing of the main pancreatic duct seen with AIP from stenosis of the main pancreatic duct seen with pancreatic carcinoma. However, MRCP findings in conjunction with MR imaging of pancreatic enlargement that shows abnormal signal intensity on T1- and T2-weighted MR images are useful in supporting a diagnosis of AIP.     

Diagnosis and management of autoimmune pancreatitis： Experience from China

AIM： To determine the clinical, radiographic and pathologic characteristics, diagnostic and treatment modalities in patients with autoimmune pancreatitis （AIP）. METHODS： In this retrospective study, the data of patients with diagnosed chronic pancreatitis （CP） between 1995 and 2006 in Chinese PLA General Hospital were included to screen for the cases with AIP, according to the following diagnostic criteria： （1） diagnostic histopathologic features, and abound IgG4-positive plasma cells on pancreatic tissues; （2） characteristic imaging on computed tomography and pancreatography, together with increased serum IgG, y-globulin levels or presence of autoantibodies; （3） response to steroid therapy. The clinical, radiographic and pathologic characteristics, diagnostic and treatment modalities, and outcome of AIP cases were reviewed. RESULTS： Twenty-five （22 male, 3 female; mean age 54 years, 36-76 years） out of 510 CP patients were diagnosed as AIP, which accounted for 49% （21/43） of CP population undergoing surgical treatment in the same period. The main clinical manifestations included intermittent or progressive jaundice in 18 cases （72%）, abdominal pain in 11 （44%）, weight loss in 10 （40%）, and 3 cases had no symptoms. The imaging features consisted of pancreatic enlargement, especially in the head of pancreas （18 cases）, strictures of main pancreatic duct and intrapancreatic bile duct. Massive lymphocytes and plasma cells infiltration in pancreatic tissues were showed on pathology, as well as parenchymal fibrosis. Twenty-three patients were misdiagnosed as pancreaticobiliary malignancy, and 21 patients underwent exploratory laparotomy, theremaining 4 patients dramatically responded to steroid therapy. No pancreatic cancer occurred during a mean 46-mo follow-up period. CONCLUSION： AIP patients always are subjected to mistaken diagnosis of pancreatic cancer and an unnecessary surgical exploration, due to its similarity in clinical features with pancreatic cancer. T     

Isolated intrapancreatic Ig G4-related sclerosing cholangitis

Immunoglobulin G4-related sclerosing cholangitis(Ig G4-SC) is frequently associated with type 1 autoimmune pancreatitis(AIP). Association with AIP can be utilized in the diagnosis of Ig G4-SC. However, some cases of Ig G4-SC are isolated from AIP, which complicates the diagnosis. Most of the reported cases of isolated Ig G4-SC displayed hilar biliary strictures, whereas isolated Ig G4-SC with intrapancreatic biliary stricture is very rare. Recently, we have encountered 5 isolated intrapancreatic Ig G4-SC cases that were not associated with AIP, three of which were pathologically investigated after surgical operation. They all were males with a mean age of 74.2 years. The pancreas was not enlarged in any of these cases. No irregular narrowing of the main pancreatic duct was found. Bile duct wall thickening in lesions without luminal stenosis was detected by abdominal computed tomography in all five cases, by endoscopic ultrasonography in two out of four cases and by intraductal ultrasonography in all three cases. In three cases, serum Ig G4 levels were within the normal limits. The mean serum Ig G4 level measured before surgery was 202.1 mg/d L(4 cases). Isolated intrapancreatic Ig G4-SC is difficult to diagnose, especially if the Ig G4 level remains normal. Thus, this type of Ig G4-SC should be suspected in addition to cholangiocarcinoma and pancreatic cancer if stenosis of intrapancreatic bile duct is present.     

Clinical implications of accessory pancreatic duct

The accessory pancreatic duct (APD) is the main drainage duct of the dorsal pancreatic bud in the embryo,entering the duodenum at the minor duodenal papilla (MIP).With the growth,the duct of the dorsal bud undergoes varying degrees of atrophy at the duodenal end.Patency of the APD in 291 control cases was 43% as determined by dye-injection endoscopic retrograde pancreatography.Patency of the APD in 46 patients with acute pancreatitis was only 17%,which was significantly lower than in control cases (P < 0.01).The terminal shape of the APD was correlated with APD patency.Based on the data about correlation between the terminal shape of the APD and its patency,the estimated APD patency in 167 patients with acute pancreatitis was 21%,which was signif icantly lower than in control cases (P < 0.01).A patent APD may function as a second drainage system for the main pancreatic duct to reduce the pressure in the main pancreatic duct and prevent acute pancreatitis.Pancreatographic f indings of 91 patients with pancreaticobiliary maljunction (PBM) were divided into a normal duct group (80 patients) and a dorsal pancreatic duct (DPD) dominant group (11 patients).While 48 patients (60%) with biliary carcinoma (gallbladder carcinoma,n=42;bile duct carcinoma,n=6) were identified in PBM with a normal pancreatic duct system,only two cases of gallbladder carcinoma (18%) occurred in DPD-dominant patients (P < 0.05).Concentration of amylase in the bile of DPD dominance was signifi cantly lower than that of normal pancreatic duct system (75 403.5 ± 82 015.4 IU/L vs 278 157.0 ± 207 395.0 IU/L,P < 0.05).In PBM with DPD dominance,most pancreatic juice in the upper DPD is drained into the duodenum via the MIP,and reflux of pancreatic juice to the biliary tract might be reduced,resulting in less frequency of associated biliary carcinoma.     

Sclerosing cholecystitis associated with autoimmune pancreatitis

AIM: To evaluate the histopathological and radiological findings of the gallbladder in patients with autoimmune pancreatitis (AIP). METHODS: The radiological findings of the gallbladder of 19 AIP patients were retrospectively reviewed. Resected gallbladders of 8 AIP patients were examined histologically and were immunostained with anti- IgG4 antibody. Controls consisted of gallbladders resected for symptomatic gallstones (n = 10) and those removed during pancreatoduodenectomy for pancreatic carcinoma (n = 10), as well as extrahepatic bile ducts and pancreases removed by pancreatoduodenectomy for pancreatic carcinoma (n = 10). RESULTS: Thickening of the gallbladder wall was detected by ultrasound and/or computed tomography in 10 patients with AIP (3 severe and 7 moderate); in these patients severe stenosis of the extrahepatic bile duct was also noted. Histologically, thickening of the gallbladder was detected in 6 of 8 (75%) patients with AIP; 4 cases had transmural lymphoplasmacytic infiltration with fibrosis, and 2 cases had mucosal-based lymphoplasmacytic infiltration. Considerable transmural thickening of the extrahepatic bile duct wall with dense fibrosis and diffuse lymphoplasmacytic infiltration was detected in 7 patients. Immunohistochemically, severe or moderate infiltration of IgG4-positive plasma cells was detected in the gallbladder, bile duct, and pancreas of all 8 patients, but was not detected in controls. CONCLUSION: Gallbladder wall thickening with fibrosis and abundant infiltration of IgG4-positive plasma cells is frequently detected in patients with AIR We propose the use of a new term, sclerosing cholecystitis, for these cases that are induced by the same mechanism as sclerosing pancreatitis or sclerosing cholangitis in AIR     

Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis

AIM： To clarify the characteristic features of biliary lesions in patients with autoimmune pancreatitis （AIP） and compare them with those of primary sclerosing cholangitis （PSC）.METHODS： The clinicopathological characteristics of 34 patients with sclerosing cholangitis （SC） associated with AIP were compared with those of 4 patients with PSC.RESULTS： SC with AIP occurred predominantly in elderly men. Obstructive jaundice was the most frequent initial symptom in SC with AIP. Only SC patients with AIP had elevated serum IgG4 levels, and sclerosing diseases were more frequent in these patients. SC patients with AIP responded well to steroid therapy. Segmental stenosis of the lower bile duct was observed only in SC patients with AIP, but a beaded and prunedtree appearance was detected only in PSC patients.Dense infiltration of IgG4-positive plasma cells was detected in the bile duct wall and the periportal area, as well as in the pancreas, of SC patients with AIP.CONCLUSION： SC with AIP is distinctly different from PSC. The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.     

Diagnosis of autoimmune pancreatitis

Autoimmune pancreatitis(AIP) is a distinct form of chronic pancreatitis that is increasingly being reported. The presentation and clinical image findings of AIP sometimes resemble those of several pancreatic malignancies, but the therapeutic strategy differs appreciably. Therefore, accurate diagnosis is necessary for cases of AIP. To date, AIP is classified into two distinct subtypes from the viewpoints of etiology, serum markers, histology, other organ involvements, and frequency of relapse: type 1 is related to Ig G4(lymphoplasmacytic sclerosing pancreatitis) and type 2 is related to a granulocytic epithelial lesion(idiopathic duct-centric chronic pancreatitis). Both types of AIP are characterized by focal or diffuse pancreatic enlargement accompanied with a narrowing of the main pancreatic duct, and both show dramatic responses to corticosteroid. Unlike type 2, type 1 is characteristically associated with increasing levels of serum Ig G4 and positive serum autoantibodies, abundant infiltration of Ig G4-positive plasmacytes, frequent extrapancreatic lesions, and relapse. These findings have led several countries to propose diagnostic criteria for AIP, which consist of essentially similar diagnostic items; however, several differences exist for each country, mainly due to differences in the definition of AIP and the modalities used to diagnose this disease. An attempt to unite the diagnostic criteria worldwide was made with the publication in 2011 of the international consensus diagnostic criteria for AIP, established at the 2010 Congress of the International Association of Pancreatology(IAP).     

Clinical significance of main pancreatic duct dilation on computed tomography： Single and double duct dilation

AIM: To study the patients with main pancreatic duct dilation on computed tomography (CT) and thereby to provide the predictive criteria to identify patients at high risk of significant diseases, such as pancreatic cancer, and to avoid unnecessary work up for patients at low risk of such diseases. METHODS: Patients with dilation of the main pancreatic duct on CT at Emory University Hospital in 2002 were identified by computer search. Clinical course and ultimate diagnosis were obtained in all the identified patients by abstraction of their computer database records. RESULTS: Seventy-seven patients were identified in this study. Chronic pancreatitis and pancreatic cancer were the most common causes of the main pancreatic duct dilation on CT. Although the majority of patients with isolated dilation of the main pancreatic duct (single duct dilation) had chronic pancreatitis, one-third of patients with single duct dilation but without chronic pancreatitis had pancreatic malignancies, whereas most of patients with concomitant biliary duct dilation (double duct dilation) had pancreatic cancer. CONCLUSION: Patients with pancreatic double duct dilation need extensive work up and careful followup since a majority of these patients are ultimately diagnosed with pancreatic cancer. Patients with single duct dilation, especially such patients without any evidence of chronic pancreatitis, also need careful follow-up since the possibility of pancreatic malignancy, including adenocarcinoma and intraductal papillary mucinous tumors, is still high.     

State of knowledge of helminth fauna of freshwater fishes of Poland

A total 89 fish and lamprey species has been recorded from Polish freshwater habitats. Twenty-seven of them (30.3%) have not been surveyed for parasitic helminthes. Some of the latter fishes are either rare or not easily accessible. Other live only in specific habitats in scattered localities. An important obstacle for studying parasite faunas of some fishes may be their status on an endangered species. Among the non-surveyed fishes, are those which have been relatively recently introduced to Poland or migrated there on their own. The present paper attempts to review all hitherto not studied helminthologically fish species, their habitats, localities and current protection status.     

高血压降压治疗目标的再认识

根据传统的高血压水平的定义,1993年WHO高血压治疗指南提出血压控制目标为<140/90mm Hg(1mm Hg=0.133kPa),但是并非所有患者都必须将血压降至同一水平,而应根据患者情况进行个体化治疗。Framingham进行的一项长达10～12年的心血管事件研究发现,第5年后,正常上限血压[收缩压(SBP