Autoimmune pancreatitis (AIP) is a newly described entity of pancreatitis in which the pathogenesis appears to involve autoimmune mechanisms. Based on histological and immunohistochemical examinations of various organ...Autoimmune pancreatitis (AIP) is a newly described entity of pancreatitis in which the pathogenesis appears to involve autoimmune mechanisms. Based on histological and immunohistochemical examinations of various organs of AIP patients, AIP appears to be a pancreatic lesion reflecting a systemic "IgG4-related sclerosing disease". Clinically, AIP patients and patients with pancreatic cancer share many features, such as preponderance of elderly males, frequent initial symptom of painless jaundice, development of new-onset diabetes mellitus, and elevated levels of serum tumor markers. It is of uppermost importance not to misdiagnose AIP as pancreatic cancer. Since there is currently no diagnostic serological marker for AIP, and approach to the pancreas for histological examination is generally difficult, AIP is diagnosed using a combination of clinical, serological, morphological, and histopathological features. Findings suggesting AIP rather than pancreatic cancer include:fluctuating obstructive jaundice; elevated serum IgG4 levels; diffuse enlargement of the pancreas; delayed en- hancement of the enlarged pancreas and presence of a capsule-like rim on dynamic computed tomography; low apparent diffusion coefficient values on diffusion-weighted magnetic resonance image; irregular narrowing of the main pancreatic duct on endoscopic retrograde cholangiopancreatography; less upstream dilatation of the main pancreatic duct on magnetic resonance cholangiopancreatography, presence of other organ involvement such as bilateral salivary gland swelling, retroperitoneal fibrosis and hilar or intrahepatic sclerosing cholangitis; negative work-up for malignancy including endoscopic ultrasound-guided fine needle aspiration; and steroid responsiveness. Since AIP responds dramatically to steroid therapy, accurate diagnosis of AIP can avoid unnecessary laparotomy or pancreatic resection.展开更多
目的探讨慢性肾衰竭维持性血液透析患者血浆致动脉硬化指数(atherogenic index of plasma, AIP)与炎性因子及心血管疾病的相关性。方法选取2014年5月—2017年12月我院收治的108例慢性肾衰竭维持性血液透析患者为研究对象,根据AIP检查结...目的探讨慢性肾衰竭维持性血液透析患者血浆致动脉硬化指数(atherogenic index of plasma, AIP)与炎性因子及心血管疾病的相关性。方法选取2014年5月—2017年12月我院收治的108例慢性肾衰竭维持性血液透析患者为研究对象,根据AIP检查结果将其分为AIP升高组(≥0.89)58例和AIP降低组(<0.89)50例,并根据随访是否发生心血管疾病分为A组(发生心血管疾病)46例和B组(未发生心血管疾病)62例;另选取同期我院体检正常者60例作为对照组。比较所有入选者AIP、炎性因子及相关临床指标水平,分析AIP与炎性因子的相关性,分析慢性肾衰竭维持性血液透析患者心血管疾病发生的危险因素。结果 AIP升高组C-反应蛋白(CRP)、肿瘤坏死因子-ɑ(TNF-ɑ)、白细胞介素-6(IL-6)显著高于AIP降低组,差异均有统计学意义(P<0.05或P<0.01)。Spearman等级相关分析结果显示,AIP与CRP、TNF-ɑ、IL-6水平呈正相关。多因素logistic回归分析结果显示,AIP、CRP、TNF-α和IL-6水平过高为慢性肾衰竭维持性血液透析患者心血管疾病发生的独立危险因素。结论慢性肾衰竭维持性血液透析患者AIP与CRP、TNF-α和IL-6水平呈正相关,且AIP、CRP、TNF-α和IL-6水平过高为慢性肾衰竭维持性血液透析患者心血管疾病发生的独立危险因素,AIP可作为预测慢性肾衰竭维持性血液透析患者心血管疾病发生的有效指标。展开更多
AIM: To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4). METHODS: The serum level of IgG4 was measured in 61 ...AIM: To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4). METHODS: The serum level of IgG4 was measured in 61 patients with SAIDs of different types who had not yet participated in glucocorticosteroid treatment. Patients with an elevated IgG4 level were examined by abdominal ultrasonography (US) and, in some cases, by computer tomography (CT). RESULTS: Elevated serum IgG4 levels (919 ± 996 mg/L) were detected in 17 (28%) of the 61 SAID patients. 10 patients had Sj gren's syndrome (SS) (IgG4: 590 ± 232 mg/L), 2 of them in association with Hashimoto's thyroiditis, and 7 patients (IgG4: 1388 ± 985.5 mg/L) had systemic lupus erythematosus (SLE). The IgG4 level in the SLE patients and that in patients with SS were not significantly different from that in AIP patients (783 ± 522 mg/L). Abdominal US and CT did not reveal any characteristic features of AIP among the SAID patients with an elevated IgG4 level. CONCLUSION: The serum IgG4 level may be elevated in SAIDs without the presence of AIP. The determination of serum IgG4 does not seem to be suitable for the differentiation between IgG4-related diseases and SAIDs.展开更多
AIM:To clarify the characteristic features of biliary le-sions in patients with autoimmune pancreatitis(AIP) and compare them with those of primary sclerosing cholangitis(PSC) .METHODS:The clinicopathological characte...AIM:To clarify the characteristic features of biliary le-sions 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 el-derly 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 pa-tients with AIP responded well to steroid therapy.Seg-mental stenosis of the lower bile duct was observed only in SC patients with AIP,but a beaded and pruned-tree appearance was detected only in PSC patients.Dense infi ltration of IgG4-positive plasma cells was de-tected 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.展开更多
AIM:To investigate differences in clinical features between diffuse-and focal-type autoimmune pancreatitis(AIP).METHODS:Based on radiological findings by computed tomography and/or magnetic resonance imaging,we divide...AIM:To investigate differences in clinical features between diffuse-and focal-type autoimmune pancreatitis(AIP).METHODS:Based on radiological findings by computed tomography and/or magnetic resonance imaging,we divided 67 AIP patients into diffuse type(D type) and focal type(F type).We further divided F type into head type(H type) and body and/or tail type(B/T type) according to the location of enlargement.Finally,we classified the 67 AIP patients into three groups:D type,H type and B/T type.We compared the three types of AIP in terms of clinical,laboratory,radiological,functional and histological findings and clinical course.RESULTS:There were 34 patients with D-type,19 with H-type and 14 with B/T-type AIP.Although obstructive jaundice was frequently detected in D-typepatients(88%) and H-type patients(68%),no B/T-type patients showed jaundice as an initial symptom(P < 0.001).There were no differences in frequency of abdominal pain,but acute pancreatitis was associated more frequently in B/T-type patients(36%) than in D-type patients(3%)(P = 0.017).Serum immunoglobulin G(IgG)4 levels were significantly higher in D-type patients(median 309 mg/dL) than in B/T-type patients(133.5 mg/dL)(P = 0.042).Serum amylase levels in B/T-type patients(median:114 IU/L) were significantly greater than in H-type patients(72 IU/L)(P = 0.049).Lymphoplasmacytic sclerosing pancreatitis(LPSP) was histologically confirmed in 6 D-type,7 H-type and 4 B/T-type patients;idiopathic duct-centric pancreatitis was observed in no patients.Marked fibrosis and abundant infiltration of CD20-positive B lymphocytes with few IgG4-positive plasma cells were detected in 2 B/T-type patients.Steroid therapy was effective in all 50 patients(31 D type,13 H type and 6 B/T type).Although AIP relapsed during tapering or after stopping steroids in 3 D-type and 3 H-type patients,no patients relapsed in B/T type.During follow-up,radiological features of 6 B/T-type patients were not changed and 1 B/T-type patient improved naturally.CONCLUSION:Clinical features of H展开更多
文摘Autoimmune pancreatitis (AIP) is a newly described entity of pancreatitis in which the pathogenesis appears to involve autoimmune mechanisms. Based on histological and immunohistochemical examinations of various organs of AIP patients, AIP appears to be a pancreatic lesion reflecting a systemic "IgG4-related sclerosing disease". Clinically, AIP patients and patients with pancreatic cancer share many features, such as preponderance of elderly males, frequent initial symptom of painless jaundice, development of new-onset diabetes mellitus, and elevated levels of serum tumor markers. It is of uppermost importance not to misdiagnose AIP as pancreatic cancer. Since there is currently no diagnostic serological marker for AIP, and approach to the pancreas for histological examination is generally difficult, AIP is diagnosed using a combination of clinical, serological, morphological, and histopathological features. Findings suggesting AIP rather than pancreatic cancer include:fluctuating obstructive jaundice; elevated serum IgG4 levels; diffuse enlargement of the pancreas; delayed en- hancement of the enlarged pancreas and presence of a capsule-like rim on dynamic computed tomography; low apparent diffusion coefficient values on diffusion-weighted magnetic resonance image; irregular narrowing of the main pancreatic duct on endoscopic retrograde cholangiopancreatography; less upstream dilatation of the main pancreatic duct on magnetic resonance cholangiopancreatography, presence of other organ involvement such as bilateral salivary gland swelling, retroperitoneal fibrosis and hilar or intrahepatic sclerosing cholangitis; negative work-up for malignancy including endoscopic ultrasound-guided fine needle aspiration; and steroid responsiveness. Since AIP responds dramatically to steroid therapy, accurate diagnosis of AIP can avoid unnecessary laparotomy or pancreatic resection.
文摘目的探讨慢性肾衰竭维持性血液透析患者血浆致动脉硬化指数(atherogenic index of plasma, AIP)与炎性因子及心血管疾病的相关性。方法选取2014年5月—2017年12月我院收治的108例慢性肾衰竭维持性血液透析患者为研究对象,根据AIP检查结果将其分为AIP升高组(≥0.89)58例和AIP降低组(<0.89)50例,并根据随访是否发生心血管疾病分为A组(发生心血管疾病)46例和B组(未发生心血管疾病)62例;另选取同期我院体检正常者60例作为对照组。比较所有入选者AIP、炎性因子及相关临床指标水平,分析AIP与炎性因子的相关性,分析慢性肾衰竭维持性血液透析患者心血管疾病发生的危险因素。结果 AIP升高组C-反应蛋白(CRP)、肿瘤坏死因子-ɑ(TNF-ɑ)、白细胞介素-6(IL-6)显著高于AIP降低组,差异均有统计学意义(P<0.05或P<0.01)。Spearman等级相关分析结果显示,AIP与CRP、TNF-ɑ、IL-6水平呈正相关。多因素logistic回归分析结果显示,AIP、CRP、TNF-α和IL-6水平过高为慢性肾衰竭维持性血液透析患者心血管疾病发生的独立危险因素。结论慢性肾衰竭维持性血液透析患者AIP与CRP、TNF-α和IL-6水平呈正相关,且AIP、CRP、TNF-α和IL-6水平过高为慢性肾衰竭维持性血液透析患者心血管疾病发生的独立危险因素,AIP可作为预测慢性肾衰竭维持性血液透析患者心血管疾病发生的有效指标。
基金Supported by Grants TáMOP-4.2.1./B-09/1/KONV and 4.2.2-08/1-2008-0002 (partly)
文摘AIM: To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4). METHODS: The serum level of IgG4 was measured in 61 patients with SAIDs of different types who had not yet participated in glucocorticosteroid treatment. Patients with an elevated IgG4 level were examined by abdominal ultrasonography (US) and, in some cases, by computer tomography (CT). RESULTS: Elevated serum IgG4 levels (919 ± 996 mg/L) were detected in 17 (28%) of the 61 SAID patients. 10 patients had Sj gren's syndrome (SS) (IgG4: 590 ± 232 mg/L), 2 of them in association with Hashimoto's thyroiditis, and 7 patients (IgG4: 1388 ± 985.5 mg/L) had systemic lupus erythematosus (SLE). The IgG4 level in the SLE patients and that in patients with SS were not significantly different from that in AIP patients (783 ± 522 mg/L). Abdominal US and CT did not reveal any characteristic features of AIP among the SAID patients with an elevated IgG4 level. CONCLUSION: The serum IgG4 level may be elevated in SAIDs without the presence of AIP. The determination of serum IgG4 does not seem to be suitable for the differentiation between IgG4-related diseases and SAIDs.
文摘AIM:To clarify the characteristic features of biliary le-sions 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 el-derly 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 pa-tients with AIP responded well to steroid therapy.Seg-mental stenosis of the lower bile duct was observed only in SC patients with AIP,but a beaded and pruned-tree appearance was detected only in PSC patients.Dense infi ltration of IgG4-positive plasma cells was de-tected 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.
基金Supported by The Research Committee of Intractable Pancreatic Diseases (Principal investigatorTooru Shimosegawa) provided by the Ministry of Health, Labour and Welfare of Japan
文摘AIM:To investigate differences in clinical features between diffuse-and focal-type autoimmune pancreatitis(AIP).METHODS:Based on radiological findings by computed tomography and/or magnetic resonance imaging,we divided 67 AIP patients into diffuse type(D type) and focal type(F type).We further divided F type into head type(H type) and body and/or tail type(B/T type) according to the location of enlargement.Finally,we classified the 67 AIP patients into three groups:D type,H type and B/T type.We compared the three types of AIP in terms of clinical,laboratory,radiological,functional and histological findings and clinical course.RESULTS:There were 34 patients with D-type,19 with H-type and 14 with B/T-type AIP.Although obstructive jaundice was frequently detected in D-typepatients(88%) and H-type patients(68%),no B/T-type patients showed jaundice as an initial symptom(P < 0.001).There were no differences in frequency of abdominal pain,but acute pancreatitis was associated more frequently in B/T-type patients(36%) than in D-type patients(3%)(P = 0.017).Serum immunoglobulin G(IgG)4 levels were significantly higher in D-type patients(median 309 mg/dL) than in B/T-type patients(133.5 mg/dL)(P = 0.042).Serum amylase levels in B/T-type patients(median:114 IU/L) were significantly greater than in H-type patients(72 IU/L)(P = 0.049).Lymphoplasmacytic sclerosing pancreatitis(LPSP) was histologically confirmed in 6 D-type,7 H-type and 4 B/T-type patients;idiopathic duct-centric pancreatitis was observed in no patients.Marked fibrosis and abundant infiltration of CD20-positive B lymphocytes with few IgG4-positive plasma cells were detected in 2 B/T-type patients.Steroid therapy was effective in all 50 patients(31 D type,13 H type and 6 B/T type).Although AIP relapsed during tapering or after stopping steroids in 3 D-type and 3 H-type patients,no patients relapsed in B/T type.During follow-up,radiological features of 6 B/T-type patients were not changed and 1 B/T-type patient improved naturally.CONCLUSION:Clinical features of H
