Focal Type Autoimmune Pancreatitis Research Articles

Diagnostic value of homogenous delayed enhancement in contrast-enhanced computed tomography images and endoscopic ultrasound-guided tissue acquisition for patients with focal autoimmune pancreatitis.

We aimed to investigate (1) promising clinical findings for the recognition of focal type autoimmune pancreatitis (FAIP) and (2) the impact of endoscopic ultrasound (EUS)-guided tissue acquisition (EUS-TA) on the diagnosis of FAIP. Twenty-three patients with FAIP were involved in this study, and 44 patients with resected pancreatic ductal adenocarcinoma (PDAC) were included in the control group. (1) Multivariate analysis revealed that homogeneous delayed enhancement on contrast-enhanced computed tomography was a significant factor indicative of FAIP compared to PDAC (90% vs. 7%, p=0.015). (2) For 13 of 17 FAIP patients (76.5%) who underwent EUS-TA, EUS-TA aided the diagnostic confirmation of AIPs, and only one patient (5.9%) was found to have AIP after surgery. On the other hand, of the six patients who did not undergo EUS-TA, three (50.0%) underwent surgery for pancreatic lesions. Homogeneous delayed enhancement on contrast-enhanced computed tomography was the most useful clinical factor for discriminating FAIPs from PDACs. EUS-TA is mandatory for diagnostic confirmation of FAIP lesions and can contribute to a reduction in the rate of unnecessary surgery for patients with FAIP.

A CT based radiomics nomogram for differentiation between focal-type autoimmune pancreatitis and pancreatic ductal adenocarcinoma.

The purpose of this study was to develop and validate an CT-based radiomics nomogram for the preoperative differentiation of focal-type autoimmune pancreatitis from pancreatic ductal adenocarcinoma. 96 patients with focal-type autoimmune pancreatitis and pancreatic ductal adenocarcinoma have been enrolled in the study (32 and 64 cases respectively). All cases have been confirmed by imaging, clinical follow-up and/or pathology. The imaging data were considered as: 70% training cohort and 30% test cohort. Pancreatic lesions have been manually delineated by two radiologists and image segmentation was performed to extract radiomic features from the CT images. Independent-sample T tests and LASSO regression were used for feature selection. The training cohort was classified using a variety of machine learning-based classifiers, and 5-fold cross-validation has been performed. The classification performance was evaluated using the test cohort. Multivariate logistic regression analysis was then used to develop a radiomics nomogram model, containing the CT findings and Rad-Score. Calibration curves have been plotted showing the agreement between the predicted and actual probabilities of the radiomics nomogram model. Different patients have been selected to test and evaluate the model prediction process. Finally, receiver operating characteristic curves and decision curves were plotted, and the radiomics nomogram model was compared with a single model to visually assess its diagnostic ability. A total of 158 radiomics features were extracted from each image. 7 features were selected to construct the radiomics model, then a variety of classifiers were used for classification and multinomial logistic regression (MLR) was selected to be the optimal classifier. Combining CT findings with radiomics model, a prediction model based on CT findings and radiomics was finally obtained. The nomogram model showed a good sensitivity and specificity with AUCs of 0.87 and 0.83 in training and test cohorts, respectively. The areas under the curve and decision curve analysis showed that the radiomics nomogram model may provide better diagnostic performance than the single model and achieve greater clinical net benefits than the CT finding model and radiomics signature model individually. The CT image-based radiomics nomogram model can accurately distinguish between focal-type autoimmune pancreatitis and pancreatic ductal adenocarcinoma patients and provide additional clinical benefits.

Radiomics based on diffusion-weighted imaging for differentiation between focal-type autoimmune pancreatitis and pancreatic carcinoma.

To evaluate the parameters of support vector machine (SVM) using imaging data generated from the apparent diffusion coefficient (ADC) to differentiate between focal-type autoimmune pancreatitis (f-AIP) and pancreatic ductal adenocarcinoma (PDAC) when using SVM based on diffusion-weighted imaging. The 2D-ADCmean and texture parameters (16 texture features × [non-filter+17 filters]) were retrospectively segmented by 2 readers in 28 patients with f-AIP and 77 patients with pathologically proven PDAC. The diagnostic accuracy of the SVM model was evaluated by receiver operating characteristic curve analysis and calculation of the area under the curve (AUC). Interreader reliability was assessed by intraclass correlation coefficient (ICC). The 2D-ADCmean and 3D-ADCmean were significantly lower in cases of f-AIP (1.10-1.15 × 10-3 mm2/s and 1.21-1.23× 10-3 mm2/s, respectively) vs PDAC (1.29-1.33 × 10-3 mm2/s and 1.41-1.43 × 10-3 mm2/s, respectively), with excellent and good interreader reliability, respectively (ICC = 0.909 and 0.891, respectively). Among the texture parameters, energy with exponential filtering yielded the highest AUC (Reader 1: 74.7%, Reader 2: 81.5%), with fair interreader reliability (ICC = 0.707). The non-linear SVM, a combination of 2D-ADCmean, object volume and exponential-energy showed an AUC value of 96.2% in the testing cohorts. Our results suggest that non-linear SVM using a combination of 2D-ADCmean, object volume, and exponential-energy may assist in differentiating f-AIP from PDAC. The radiomics based on an apparent diffusion coefficient value may assist in differentiating f-AIP from PDAC.

The effect of CT texture-based analysis using machine learning approaches on radiologists' performance in differentiating focal-type autoimmune pancreatitis and pancreatic duct carcinoma

PurposeTo develop a support vector machine (SVM) classifier using CT texture-based analysis in differentiating focal-type autoimmune pancreatitis (AIP) and pancreatic duct carcinoma (PD), and to assess the radiologists’ diagnostic performance with or without SVM.Materials and methodsThis retrospective study included 50 patients (20 patients with focal-type AIP and 30 patients with PD) who underwent dynamic contrast-enhanced CT. Sixty-two CT texture-based features were extracted from 2D images of the arterial and portal phase CTs. We conducted data compression and feature selections using principal component analysis (PCA) and produced the SVM classifier. Four readers participated in this observer performance study and the statistical significance of differences with and without the SVM was assessed by receiver operating characteristic (ROC) analysis.ResultsThe SVM performance indicated a high performance in differentiating focal-type AIP and PD (AUC = 0.920). The AUC for all 4 readers increased significantly from 0.827 to 0.911 when using the SVM outputs (p = 0.010). The AUC for inexperienced readers increased significantly from 0.781 to 0.905 when using the SVM outputs (p = 0.310). The AUC for experienced readers increased from 0.875 to 0.912 when using the SVM outputs, however, there was no significant difference (p = 0.018).ConclusionThe use of SVM classifier using CT texture-based features improved the diagnostic performance for differentiating focal-type AIP and PD on CT.

Application of dynamic contrast enhanced ultrasound in distinguishing focal-type autoimmune pancreatitis from pancreatic ductal adenocarcinoma.

To explore the value of dynamic contrast enhanced ultrasound (DCE-US) in preoperative differential diagnosis of focal-type autoimmune pancreatitis (AIP) and pancreatic ductal adenocarcinoma (PDAC). From May 2016 to March 2020, patients with biopsy and histopathologically confirmed focal-type AIP (n = 9) were retrospectively included. All patients received contrast enhanced ultrasound (CEUS) examinations one week before surgery/biopsy. Dynamic analysis was performed by VueBox® software (Bracco, Italy). Eighteen cases of resection and histopathologically proved PDAC lesions were also included as control group. B mode ultrasound (BMUS) features, CEUS enhancement patterns, time intensity curves (TICs) and CEUS quantitative parameters were obtained and compared between AIP and PDAC lesions. After injection of ultrasound contrast agents, most focal-type AIP lesions displayed hyper-enhancement (2/9, 22.2%) or iso-enhancement (6/9, 66.7%) during arterial phase of CEUS, while most of PDAC lesions showed hypo-enhancement (88.9%) (P < 0.01). During late phase, most of AIP lesions showed iso-enhancement (8/9, 88.9%), while most of PDAC lesions showed hypo-enhancement (94.4%) (P < 0.001). Compared with PDAC lesions, TICs of AIP lesions showed delayed and higher enhancement. Among all CEUS perfusion parameters, ratio of PE (peak enhancement), WiAUC (wash-in area under the curve), WiR (wash-in rate), WiPI (wash-in perfusion index, WiPI = WiAUC/ rise time), WoAUC (wash-out area under the curve), WiWoAUC (wash-in and wash-out area under the curve) and WoR (wash-out rate) between pancreatic lesion and surrounding normal pancreatic tissue were significantly higher in AIP lesions than PDAC lesions (P < 0.05). DCE-US with quantitative analysis has the potential to make preoperative differential diagnosis between focal-type AIP and PDAC non-invasively.

CT Radiomics Features in Differentiation of Focal-Type Autoimmune Pancreatitis from Pancreatic Ductal Adenocarcinoma: A Propensity Score Analysis

To evaluate the diagnostic performance of the radiomics score (rad-score) for differentiating focal-type autoimmune pancreatitis (fAIP) from pancreatic ductal adenocarcinoma (PDAC). This retrospective review included 42 consecutive patients with fAIP diagnosed according to the International Consensus Diagnostic Criteria between January 2011 and December 2018. Furthermore, 334 consecutive patients with PDAC confirmed by pathology were also reviewed during the same period. Patients with PDAC and fAIP were matched via propensity score matching (PSM). All patients underwent multidetector computed tomography (MDCT). For each patient, 1409 radiomics features of the portal phase were extracted and reduced using the least absolute shrinkage and selection operator (LASSO) logistic regression algorithm. The portal rad-score performance was assessed based on its discriminative ability. After PSM, we matched 55 patients with PDAC to 42 patients with fAIP, based on clinical and CT characteristics (e.g., patient age, sex, body mass index, location, size, enhanced mode). A rad-score for discriminating fAIP from PDAC, which contained four CT derived radiomic features, was developed (area under the curve=0.97). The sensitivity, specificity, and accuracy of the radiomics model were 95.24%, 92.73% and 0.94, respectively. The portal rad-score can accurately and noninvasively differentiate fAIP from PDAC.

Role of Endoscopic Ultrasonography-Guided Fine Needle Aspiration/Biopsy in the Diagnosis of Autoimmune Pancreatitis

Type 1 autoimmune pancreatitis (AIP) is histologically characterized by lymphoplasmacytic sclerosing pancreatitis (LPSP). Recently, the diagnostic yield of endoscopic ultrasonography-guided fine needle aspiration/biopsy (EUS-FNA/B) for AIP has been reported. However, its role in the diagnostic flow of AIP is not fully elucidated. We retrospectively reviewed 53 consecutive patients who were suspected with AIP and underwent EUS-FNA/B. We evaluated the contribution of EUS-FNA/B to the diagnosis of AIP before considering response to steroid therapy among patients with diffuse enlargement of the pancreas and those with focal enlargement, respectively. Twenty-two patients showed diffuse pancreatic enlargement and 31 showed focal enlargement. The final diagnosis was definitive AIP in 32 patients, probable AIP in 2, possible AIP in 1, and mass-forming focal pancreatitis in 18. There was no change in diagnosis after EUS-FNA/B among patients with diffuse pancreatic enlargement, while diagnosis changed in 38.7% (12/31) among those with focal enlargement—there was a probable to definitive diagnosis in 4 patients, unspecified to definitive in 3, and unspecified to probable in 5. EUS-FNB provided a significantly higher sensitivity for typical pathological findings of LPSP than EUS-FNA, and 10 patients were diagnosed as pathologically definitive AIP by EUS-FNB, though none were by EUS-FNA (p = 0.002). EUS-FNA/B was useful in the diagnosis of focal type AIP, and steroid therapy could be introduced after the diagnosis was confirmed. Meanwhile, EUS-FNA/B provided no contribution to diagnosis of diffuse type AIP. EUS-FNB showed a higher diagnostic yield than FNA.

Differentiation of Focal-Type Autoimmune Pancreatitis From Pancreatic Ductal Adenocarcinoma Using Radiomics Based on Multiphasic Computed Tomography.

The aim of this study was to develop a radiomics model for a differential diagnosis of focal-type autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma. A total of 96 patients, 45 with AIP and 51 with pancreatic ductal adenocarcinoma, were retrospectively evaluated. All patients underwent pretreatment abdominal computed tomography imaging acquired at noncontrast, arterial, and venous phases. Furthermore, 1160 radiomics features were extracted from each phasic image to build radiomics models. The performance of radiomics model was evaluated by sensitivity, specificity, and accuracy. The results of radiomics model were also compared with those of radiologists' visual assessments. The sensitivity, specificity, and accuracy of the optimal radiomics model were 93.3%, 96.1%, and 94.8%, respectively. They were higher than those of the radiologists' assessments with sensitivity of 57.78% and 73.33%, specificity of 88.24% and 90.20%, and accuracy of 75.00% and 81.25%, respectively. Radiomics is helpful for a differential diagnosis of AIP in clinical practice as a noninvasive and quantitative method.

Endoscopic Ultrasound Features of Autoimmune Pancreatitis: A Unique Chronic Pancreatitis Pattern

Background: Autoimmune pancreatitis (AIP) is a special form of chronic pancreatitis (CP) with dramatic response to corticosteroid therapy. This study focused on the endoscopic ultrasound (EUS) features of newly diagnosed AIP. Methods: This retrospective single-center study included 286 patients of newly diagnosed AIP following the HISORt criteria, and all EUS was accomplished before corticosteroid therapy. The Rosemont criteria was applied to all the AIP patients not only for descriptions of CP features but also for diagnosis of CP. We also compared the EUS findings and the diagnosis of CP between the diffuse and focal types of AIP cases. Findings: For parenchymal changes of CP, hyperechoic foci, cystic lesion, parenchymal calcification, lobularity with/without honeycombing were all comparable between both groups, while hyperechoic striation was significantly more in the focal group (P = 0·02); for main pancreatic duct (MPD) changes of CP, MPD stones, diffuse irregularity and hyperechoic ductal margin were comparable in both groups, while MPD dilation was significantly more in the focal group (P = 0·03). A unique EUS characteristic of AIP parenchymal changes was noticed that we coined as the snowflake sign which was seen in a large number of AIP case (88·1%). The diagnosis of CP according to the Rosemont criteria was also comparable in both groups (P = 0·54). Interpretation: This study demonstrated that the newly diagnosed AIP patients showed unique CP EUS features with the snowflake sign seen in a large number of AIP cases; the diagnosis of CP according to the Rosemont criteria in the diffuse type and focal type AIP cases was comparable. Funding Statement: The authors stated: None. Declaration of Interests: The authors declare no competing interests. Ethics Approval Statement: The study was approved by the Ethics Committee of our institution, and written consent was obtained in all patients.

A feasible CT feature to differentiate focal-type autoimmune pancreatitis from pancreatic ductal adenocarcinoma.

BackgroundTo investigate whether the relative computed tomography (CT) value (rCT) of adjacent pancreatic parenchyma can distinguish focal‐type autoimmune pancreatitis (fAIP) from pancreatic ductal adenocarcinoma (PDAC).MethodsA total of 13 patients with fAIP and 20 patients with PDAC were included in this study. The rCT was calculated as the ratio of the CT value of adjacent pancreatic parenchyma to that of muscle. The diagnostic performance of rCT for discriminating fAIP from PDAC was evaluated using receiver operating characteristic (ROC) analysis.ResultsBoth fAIP and PDAC presented hyper‐fibrosis histologically and delayed enhancement on CT examination. Moreover, the pancreatic parenchyma of fAIP presented serious inflammation. The mean rCT of the parenchyma was significantly lower in fAIP than in PDAC in all phases. The best diagnostic performance of the rCT value was found in the pancreatic phase, with an area under the ROC curve of 0.912, while the areas under the ROC curve of the portal and delayed phases were 0.812 and 0.754, respectively. The optimal cut‐off value for distinguishing fAIP from PDAC was 1.62 in the pancreatic phase.ConclusionsThe rCT of the pancreatic parenchyma during the pancreatic phase may be a feasible CT feature for differentiating fAIP from PDAC.

Differentiation of focal-type autoimmune pancreatitis from pancreatic carcinoma: assessment by multiphase contrast-enhanced CT.

To evaluate the utility of multiphase contrast-enhanced computed tomography (CT) findings alone and in combination for differentiating focal-type autoimmune pancreatitis (f-AIP) from pancreatic carcinoma (PC). The study group comprised 22 f-AIP lesions and 61 PC lesions. Two radiologists independently evaluated CT findings. Frequencies of findings were compared between f-AIP and PC. Statistical, univariate and multivariate analyses were performed. Homogeneous enhancement during the portal phase (AIP, 59 % vs. PC, 3 %; P < 0.001), dotted enhancement during the pancreatic phase (50 % vs. 7 %; P < 0.001), duct-penetrating sign (46 % vs. 2 %; P < 0.001), enhanced duct sign (36 % vs. 2 %; P < 0.001) and capsule-like rim (46 % vs. 3 %; P < 0.001) were more frequently observed in AIP. Ring-like enhancement during the delayed phase (5 % vs. 46 %; P < 0.001) and peripancreatic strands with a length of at least 10 mm (5 % vs. 39 %; P = 0.001) were more frequently observed in PC. AIP was identified with 82 % sensitivity and 98 % specificity using four of these seven findings. Multivariate analysis revealed significant differences in dotted enhancement (P = 0.004), duct-penetrating sign (P < 0.001) and capsule-like rim (P = 0.007). The combination of CT findings may allow improvements in differentiating f-AIP from PC. • f-AIP can mimic PC on imaging findings. • The differentiation of f-AIP from PC is important in patient management. • Some CT findings can be used to identify AIP. • The combination of CT findings will improve differentiation from PC.

Comparison and validation of International Consensus Diagnostic Criteria for diagnosis of autoimmune pancreatitis from pancreatic cancer in a Taiwanese cohort

ObjectivesThe International Consensus Diagnostic Criteria (ICDC) designed to diagnosis autoimmune pancreatitis (AIP) has been proposed recently. The diagnostic performance of ICDC has not been previously evaluated in diffuse-type and focal-type...

Increase diagnostic accuracy in differentiating focal type autoimmune pancreatitis from pancreatic cancer with combined serum IgG4 and CA19-9 levels

Background/objectivesTo distinguish autoimmune pancreatitis (AIP), especially focal type, from pancreatic cancer, is a greatest challenge for clinician. The aim of the study is to compare the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of combined serum IgG4 and CA19-9 levels to differentiate AIP from pancreatic cancer by HISORt, Asian and international consensus diagnostic criteria. MethodsWe measured serum IgG4, CEA, and CA19-9 levels in 188 AIP patients, 86 non-AIP chronic pancreatitis patients, and 130 pancreatic cancer patients. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy were compared with different diagnostic criteria. We also compared the diagnostic performance in patients with or without jaundice. ResultsThe serum level of IgG4 was significantly higher in AIP than those in non-AIP chronic pancreatitis and pancreatic cancer. The optimal cutoffs of IgG4 and CA19-9 to differentiate AIP from pancreatic cancer were 175 mg/dL and 85.0 U/ml based on ROC analysis. Combining IgG4 level over 280 mg/dL and CA19-9 below 85.0 U/ml could yield a best diagnostic accuracy (85.6%) to distinguishing AIP from pancreatic cancer in all of the HISORt, Asian and international consensus diagnostic criteria. With the combination of serological test, focal type AIP could be diagnosed with comparable accuracy as diffuse type AIP. ConclusionsOur study demonstrated that combined use of serum IgG4 (over 280 mg/dL) and CA19-9 9 (below 85.0 U/ml) together increases the diagnostic accuracy to distinguish AIP from pancreatic cancer non-invasively, especially in focal type autoimmune pancreatitis.

Validation of international consensus diagnostic criteria for focal type autoimmune pancreatitis in a Taiwanese cohort

Peter Macinga , Adela Pulkertova , Jana Maluskova , Martin Oliverius , Julius Spicak , Tomas Hucl a a Institute for Clinical and Experimental Medicine, Department of Hepatogastroenterology, Czech Republic b Institute for Clinical and Experimental Medicine, Department of Clinical and Transplant Pathology, Czech Republic c Institute for Clinical and Experimental Medicine, Department of Transplant Surgery, Czech Republic

Increase diagnostic accuracy in differentiating focal type autoimmune pancreatitis from pancreatic cancer with combined serum IgG4 and CA19-9 levels

Increase diagnostic accuracy in differentiating focal type autoimmune pancreatitis from pancreatic cancer with combined serum IgG4 and CA19-9 levels

Peripancreatic vascular involvements of autoimmune pancreatitis

Although peripancreatic vascular lesions are occasionally encountered in autoimmune pancreatitis (AIP), there are few reports focusing on these involvements. We aimed to investigate the peripancreatic vascular involvements associated with AIP. We retrospectively analyzed 54 AIP patients who met the International Consensus Diagnostic Criteria for AIP between July 2003 and October 2010. All of the 54 patients were subjected to multiphasic multidetector computed tomography, and the prevalence, location and prognosis of peripancreatic vascular involvements were investigated. Of the 54 AIP patients, 24 (44.4%) exhibited involvements in the form of peripancreatic vascular lesions (stenoses of the splenic vein in 22 and of the superior mesenteric-portal vein in 13, development of perigastric collateral circulation in 18, gastric varices with a red color sign in one and thrombosis inside the portal vein in one). Diffuse-type AIP was associated with a significantly higher prevalence of vascular involvements compared with focal-type AIP (P = 0.033). A total of 14 out of 16 patients who underwent corticosteroid treatment showed improvement in vascular lesions. One case followed up without corticosteroid treatment and presenting an obstruction of the splenic vein exhibited involvements in the form of an infarction and hemorrhagic cysts of the spleen and ultimately underwent distal pancreatectomy and splenectomy. Autoimmune pancreatitis patients show a high prevalence of peripancreatic vascular involvements. Thus, patients with vascular involvements are suitable candidates for steroid therapy with evaluation of its potential merits and demerits, even if they are asymptomatic.

Differences between diffuse and focal autoimmune pancreatitis

To investigate differences in clinical features between diffuse- and focal-type autoimmune pancreatitis (AIP). 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. 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-type patients (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. Clinical features of H-type AIP were similar to those of D-type, but B/T-type differed from D and H types. B/T-type may involve diseases other than LPSP.

Autoimmune Pancreatitis: Differences Between the Focal and Diffuse Forms in 87 Patients

Autoimmune pancreatitis (AIP) is a particular type of chronic pancreatitis that can be classified into diffuse and focal forms. The aim of this study was to analyze clinical and instrumental features of patients suffering from the diffuse and focal forms of AIP. AIP patients diagnosed between 1995-2008 were studied. A total of 87 AIP patients (54 male and 33 female patients, mean age 43.4+/-15.3 years) were studied. Focal-type AIP was diagnosed in 63% and diffuse-type in 37%. Association with autoimmune diseases was observed in 53% of cases, the most common being ulcerative colitis (30%). Serum levels of IgG4 exceeded the upper normal limits (135 mg/dl) in 66% of focal AIP and in 27% of diffuse AIP (P=0.006). All patients responded to steroids. At recurrence non-steroid immunosuppressive drugs were successfully used in six patients. Recurrences were observed in 25% of cases, and were more frequent in focal AIP (33%) than in diffuse AIP (12%) (P=0.043), in smokers than in non-smokers (41% vs. 15%; P=0.011), and in patients with pathological serum levels of IgG4 compared to those with normal serum levels (50% vs. 12%; P=0.009). In all, 23% of the patients underwent pancreatic resections. Among patients with focal AIP, recurrences were observed in 30% of operated and in 34% of not operated patients. Focal-type and diffuse-type AIP differ as regards clinical symptoms and signs. Recurrences occur more frequently in focal AIP than in diffuse AIP. The use of non-steroid immunosuppressants may be a therapeutic option in relapsing AIP.

Incidence of the focal type of autoimmune pancreatitis in chronic pancreatitis suspected to be pancreatic carcinoma: Experience of a single tertiary cancer center

Objective. With an increase in autoimmune pancreatitis (AIP) being reported, the focal type of AIP, which shows localized narrowing of the main pancreatic duct and focal swelling of the pancreas, has recently been recognized. Therefore, cases of focal-type AIP subjected to surgical intervention for presumptive malignancy might previously have been diagnosed as mass-forming chronic pancreatitis. The aim of this study was to elucidate the incidence of focal-type AIP in resected chronic pancreatitis at a single tertiary cancer center. The clinical and radiological features of focal-type AIP were also evaluated. Material and methods. We re-evaluated 15 patients who underwent pancreatic resection with a presumed diagnosis of pancreatic ductal adenocarcinoma, and who in the past had been diagnosed pathologically as having chronic pancreatitis. Results. Seven of 15 patients showed AIP, and the other 8 patients were diagnosed as having mass-forming chronic pancreatitis not otherwise specified by pathological retrospective examination. In other words, nearly half of the cases of resected chronic pancreatitis that were suspected to be pancreatic carcinoma preoperatively showed focal-type AIP. Regarding the characteristic findings of focal-type AIP, narrowing of the pancreatic duct on endoscopic retrograde pancreatography (ERP) might be diagnostic. Conclusions. Focal-type AIP is not a rare clinical entity and might be buried in previously resected pancreatic specimens that in the past were diagnosed simply as mass-forming pancreatitis.

Clinical and imaging features of autoimmune pancreatitis with focal pancreatic swelling or mass formation: comparison with so-called tumor-forming pancreatitis and pancreatic carcinoma

Objectives Autoimmune pancreatitis (AIP) with a mass formation or swollen pancreas located in one or two segments of the gland (focal type AIP) has been reported. The aims of this study were to elucidate the relationship of the disease entity between this focal variant and so-called tumor-forming pancreatitis (TFP) and to describe the clinical and imaging features discriminating focal AIP from pancreatic carcinoma (Pca). Methods The clinical, radiologic, and pathologic profiles of nine patients with focal AIP were reviewed retrospectively and compared with those of 11 patients with alcohol-induced TFP and 80 patients with Pca. Results The patients with focal AIP were predominantly older (mean age 64.7 ± 13.6 yr, range 28–78 yr), male, and presenting with obstructive jaundice or focal pancreatic enlargement accompanied by mild abdominal symptoms. In comparison, the patients with alcohol-induced TFP who were mostly middle-aged (mean age 50.1 ± 7.95 yr, range 39–62 yr), male, and often had attacks of pancreatitis associated with findings of CT scans showing pseudocysts or peripancreatic effusion. Focal AIP usually demonstrated no abnormalities on pancreatograms downstream from the stricture or obstruction and often presented few contrast-filled side branches in the area of main pancreatic duct (MPD) stenosis. These characteristics were similar to the imaging features of Pca. Significant factors differentiating focal AIP from Pca were lower serum levels of CA19-9, homogeneous delayed enhancement evident in dynamic CT scans, and ERCP findings exhibiting a longer stenosed MPD and a thinner MPD upstream from the stricture. Conclusions Focal AIP is associated with clinical and radiologic features that are different from those of alcohol-induced TFP. In TFP there are two causative factors, namely, AIP and alcohol-induced chronic pancreatitis. Differential diagnosis of focal AIP from Pca seems to be possible in many cases by evaluating imaging findings such as dynamic CT and ERCP, although focal AIP sometimes shows clinical and radiologic features similar to those of Pca.