Durability and outcome of endoscopic ultrasound-guided hepaticoduodenostomy using a fully covered metal stent for segregated right intrahepatic duct dilatation.

BACKGROUND AND AIMS: Segregated right intrahepatic duct dilatation (IHD) results from complete obstruction of the biliary tract proximal to the hilar level. We aimed to evaluate long-term efficacy and safety of endoscopic ultrasound (EUS) hepaticoduodenostomy (HDS) in segregated right IHD. METHODS: Consecutive patients who had undergone EUS-guided HDS with a fully covered self-expandable metal stent (FCSEMS) in an academic tertiary center were recruited. All patients had segregated right hepatic duct and failed drainage by endoscopic retrograde cholangiopancreatography (ERCP). Demographic data, endoscopic findings, procedure details, and outcome data were extracted from a prospectively maintained database. RESULTS: From 2013 to 2017, there were 35 patients who had undergone EUS-guided HDS with a median follow-up duration of 169 (3-2091) days. Malignancy accounted for 71.4% of the ductal segregation, followed by surgical complication (17.1%). Technical and clinical success rate was 97.1% and 80%, respectively. Early adverse event (AE) happened in seven patients (20%), two of them required endoscopic reintervention, and no percutaneous transhepatic biliary drainage (PTBD) or surgery was performed because of AE. The median stent patency duration was 331 (3-1202) days. The median duration of fistula tract keeping was 1280 (3-1280) days. There was no significant difference in terms of patency rate with respect to whether the underlying pathology was benign or malignant (P = 0.776). EUS-guided HDS for right posterior sectional duct segregation was associated with higher 3-month stent patency rate when compared with right anterior sectional duct (79.1% vs 38.1%, P = 0.012). CONCLUSION: Endoscopic ultrasound-guided HDS with an FCSEMS appears to be a safe and effective treatment as a viable alternative option to PTBD after failed ERCP. It creates a durable and reliable fistula tract for permanent access to an isolated ductal system, and this application deserves more attention.

EUS-guided cholecystostomy versus endoscopic transpapillary cholecystostomy for acute cholecystitis in high-risk surgical patients.

BACKGROUND AND AIMS: Endoscopic gallbladder drainage (GBD) has been performed as an alternative to percutaneous drainage for acute cholecystitis. To date, there has been no comparative study between EUS-guided cholecystostomy (EUSC) and endoscopic transpapillary cholecystostomy (ETC). The aim of this study was to compare the outcomes of EUSC and ETC. METHODS: A retrospective review of an endoscopic GBD database prospectively collected at the Asan Medical Center (between July 2010 and December 2014) was performed to identify consecutive patients with acute cholecystitis who underwent attempted endoscopic GBD. Procedural and long-term outcomes were evaluated using inverse probability of treatment weighting (IPTW). RESULTS: A total of 172 patients (76 in the EUSC group and 96 in the ETC group) were included in this study. Seven patients who failed to undergo ETC crossed over to the EUSC group. After adjustment with the IPTW method, technical success (99.3% vs 86.6%, P < .01) and clinical success (99.3% vs 86%, P < .01) rates were significantly higher in the EUSC group than in the ETC group. The procedure-related adverse event rate was significantly higher in the ETC group (7.1% vs 19.3%, P = .02). The cholecystitis or cholangitis recurrence rate (12.4% vs 3.2%) was also higher in the ETC group than in the EUSC group, as identified using Cox analysis (hazard ratio, 3.01; 95% confidence interval, .73-12.9; P = .04). CONCLUSIONS: In patients with acute cholecystitis who are unfit for surgery, EUSC may be a more suitable treatment method than ETC.

Feasibility and safety of endoscopic ultrasound-guided gallbladder drainage using a newly designed lumen-apposing metal stent.

BACKGROUND AND AIMS: Endoscopic ultrasound-guided gallbladder drainage (EUS-GBD) is increasingly accepted as an effective treatment option in patients who require drainage for acute cholecystitis. A newly designed lumen-apposing metal stent (LAMS) has been introduced recently in this procedure. In this study, we evaluated the feasibility and safety of the newly designed LAMS in patients with acute cholecystitis who were unsuitable for cholecystectomy. METHODS: Between Mar 2017 and Oct 2017, 22 patients with acute cholecystitis who were unsuitable for cholecystectomy underwent EUS-GBD with the newly designed LAMS. We evaluated the technical and clinical success and the adverse event profiles. RESULTS: EUS-GBD with newly designed LAMS was technically and clinically successful in 21 of the 22 patients. EUS-GB stenting was performed at urgent setting in 17 patients, while 5 patients, who had undergone initial PTGBD, underwent EUS-GB stenting to remove PTGBD tube. The median procedure time was 11.5 (range 8.8-17.0) min. A late adverse event of stent occlusion developed in one patient. Stent migration was not observed during follow-up (median 318.0 days, range 39.0-398.0 days) and cumulative stent patency rate at 1 year was 95%. CONCLUSION: EUS-GBD with newly designed LAMS is feasible and shows acceptable safety profiles for both the urgent drainage of acute cholecystitis and elective internalization following PTGBD in patients with high surgical risk.

Comparison between groove carcinoma and groove pancreatitis.

BACKGROUND/OBJECTIVES: The pancreatoduodenal groove (anatomical groove) is a potential space bordered by the head of the pancreas, duodenum, and common bile duct. Discerning between groove carcinoma (GC) and groove pancreatitis (GP) is often difficult, but clinically important. We retrospectively analyzed and compared the findings of computed tomography (CT), laboratory tests, and endoscopic ultrasound-fine needle aspiration (EUS-FNA) for GC and GP. METHODS: GC (n = 36) and GP (n = 44) patients at Asan Medical Center from January 1, 2000, to May 31, 2017 were retrospectively reviewed. MDCT findings, baseline characteristics, laboratory test results, and EUS and EUS-FNA findings of GC and GP patients were compared. RESULTS: CT showed no significant difference in groove enhancement between the groups. Mass-like lesions, cystic groove lesions, and calcification were observed in 86.1% and 15.9%, 38.9% and 75%, and 2.8% and 29.5% of GC and GP patients, respectively. Patients were tested for total bilirubin (GC: 2.0 vs. GP: 0.6 mg/dL), cancer antigen 19-9 (CA19-9) (GC: 76 vs. GP: 12.5 U/mL), and carcinoembryonic antigen (GC: 2.4 vs. GP: 2 ng/mL). Three GP patients died, and one GP patient was diagnosed with GC. However, among 30 GC patients with at least 1-year follow-up, 20 died. In multivariate logistic regression, CA19-9, and mass-like lesion on multidetector CT (MDCT) were discriminating factors between GC and GP. Among 23 (10 GC, 13 GP) patients who underwent EUS-FNA, the diagnostic sensitivity, specificity, positive predictive value, negative predictive value, accuracy of EUS-FNA were 90%, 100%, 100%, 92.86%, and 95.65%, respectively. CONCLUSIONS: Several MDCT and laboratory findings favor GC over GP. EUS-FNA should be considered in patients with elevated CA19-9 levels and mass-like lesions on MDCT.

Usefulness of argon plasma coagulation ablation subsequent to endoscopic snare papillectomy for ampullary adenoma.

BACKGROUND AND AIM: Endoscopic snare papillectomy (ESP) is an effective treatment for ampullary adenoma. Argon plasma coagulation (APC) is widely used as an additional method to control bleeding or ablate the residual tumor. However, the efficacy of this procedure has not yet been fully evaluated. This study aimed to evaluate the usefulness of APC as an additional method to ESP. METHODS: Patients who underwent ESP for ampullary adenoma between September 2005 and September 2015 were retrospectively reviewed. Using propensity score matching, we compared short- and long-term outcomes between the ESP-with-additional-APC group (ESP + APC group) and the ESP-only group. Primary outcome was early post-ESP adverse events (AE), and secondary outcomes were late AE and recurrence. RESULTS: Among 109 patients, additional APC was carried out in 59 (54.1%) patients. After matching, 41 patients were included in both groups, respectively. Bleeding rate was significantly lower in the ESP + APC group than in the ESP-only group (7.3% vs 31.7%, odds ratio = 0.180, P < 0.01). However, there were no significant differences in other procedure-related early AE such as pancreatitis (12.2% vs 19.5%, P = 0.365), cholangitis (2.4% vs 9.8%, P = 0.198), and perforation (2.4% vs 2.4%, P = 1.000) between the ESP + APC group and the ESP-only group. During the follow-up period (mean 904 ± 868 days), papillary stricture (9.8% vs 4.9%, P = 0.405) and recurrence rates (24.4% vs 24.4%, P = 0.797) were not significantly different between the ESP + APC group and the ESP-only group. CONCLUSION: Additional APC during ESP may have a beneficial effect by decreasing bleeding rate without harmful effects.

Revised Atlanta classification and determinant-based classification: Which one better at stratifying outcomes of patients with acute pancreatitis?

BACGROUND: Two different severity classifications (revised Atlanta classification [RAC] and determinant-based classification [DBC]) were recently proposed. METHODS: This was a retrospective analysis of a prospective acute pancreatitis (AP) database. This study aims to compare the ability of three classification systems (RAC, DBC, and original Atlanta classification [OAC]) to stratify outcomes of AP and to determine the association between different severity categories and clinical outcomes. RESULTS: Overall, as the grade of severity increased, the morbidity and mortality increased accordingly in the three classification systems. The RAC and DBC were comparable, but performed better than OAC in predicting mortality (AUC 0.92 and 0.95 vs. 0.66, p < 0.001), ICU admission (AUC 0.92 and 0.96 vs. 0.68, p < 0.001), ICU LOS (AUC 0.73 and 0.76 vs. 0.50, p < 0.001), and hospital stay (AUC 0.81 and 0.83 vs. 0.70, p < 0.001). The DBC performed better than the RAC and OAC in predicting the need for intervention (AUC 0.87 vs. 0.79 and 0.68, p < 0.05). The mortality rate in patients with critical DBC category was higher than that in those with severe RAC category (42.1% vs. 24.7%; p = 0.008). POF (OR 19.4, p = 0.001) and IN (OR 11.0, p = 0.025) were independent risk factors for mortality. CONCLUSIONS: In tertiary referral setting, patients in the critical category are at the greatest risk for death and should be managed in an intensive care unit. Although IN itself may be less influential on mortality than POF, IN as well as POF should be considered as the key determinants for severity stratification.

Long-term outcomes of a newly developed hybrid metal stent for EUS-guided biliary drainage (with videos).

BACKGROUND AND AIMS: Although fully covered self-expandable metal stents (FCSEMSs) have been commonly used for EUS-guided biliary drainage (EUS-BD), FCSEMS migration is a main limitation of this procedure. In the present study we evaluated the technical and clinical success rates, adverse events, and long-term outcomes of a newly developed hybrid stent that has been customized for EUS-BD. METHODS: From September 2011 to May 2015, 54 consecutive patients with biliary obstruction were enrolled in this prospective, observational study. These patients were candidates for alternative BD techniques because of failed ERCP. The hybrid metal stent used for EUS-BD in this study was partially covered, had anchoring flaps, and is commercially available in Korea. RESULTS: EUS-guided hepaticogastrostomy (EUS-HGS) was performed in 21 patients and EUS-guided choledochoduodenostomy (EUS-CDS) in 33 patients. The technical and clinical success rates of EUS-BD were 100% (54/54) and 94.4% (51/54), respectively. Immediate adverse events developed after EUS-BD in 9 patients (16.6%; cholangitis in 3, bleeding in 2, self-limited pneumoperitoneum in 3, and abdominal pain in 1). Proximal or distal stent migration was not observed during the follow-up period (median, 148.5 days; IQR, 79.7-244), and the mean stent patency duration was 166.3 days and 329.1 days in the EUS-HGS and EUS-CDS groups, respectively. CONCLUSIONS: EUS-BD with the hybrid metal stent is technically feasible and can effectively treat biliary obstruction after failed ERCP. EUS-BD with the hybrid metal stent can reduce stent-related adverse events, especially stent migration.

Factors and outcomes associated with pancreatic duct disruption in patients with acute necrotizing pancreatitis.

BACKGROUND AND AIMS: Acute necrotizing pancreatitis (ANP) can affect main pancreatic duct (MPD) as well as parenchyma. However, the incidence and outcomes of MPD disruption has not been well studied in the setting of ANP. METHODS: This retrospective study investigated 84 of 465 patients with ANP who underwent magnetic resonance cholangiopancreatography and/or endoscopic retrograde cholangiopancreatography. The MPD disruption group was subclassified into complete and partial disruption. RESULTS: MPD disruption was documented in 38% (32/84) of the ANP patients. Extensive necrosis, enlarging/refractory pancreatic fluid collections (PFCs), persistence of amylase-rich output from percutaneous drainage, and amylase-rich ascites/pleural effusion were more frequently associated with MPD disruption. Hospital stay was prolonged (mean 55 vs. 29 days) and recurrence of PFCs (41% vs. 14%) was more frequent in the MPD disruption group, although mortality did not differ between ANP patients with and without MPD disruption. Subgroup analysis between complete disruption (n = 14) and partial disruption (n = 18) revealed a more frequent association of extensive necrosis and full-thickness glandular necrosis with complete disruption. The success rate of endoscopic transpapillary pancreatic stenting across the stricture site was lower in complete disruption (20% vs. 92%). Patients with complete MPD disruption also showed a high rate of PFC recurrence (71% vs. 17%) and required surgery more often (43% vs. 6%). CONCLUSIONS: MPD disruption is not uncommon in patients with ANP with clinical suspicion on ductal disruption. Associated MPD disruption may influence morbidity, but not mortality of patients with ANP. Complete MPD disruption is often treated by surgery, whereas partial MPD disruption can be managed successfully with endoscopic transpapillary stenting and/or transmural drainage. Further prospective studies are needed to study these items.

Severe acute pancreatitis due to tamoxifen-induced hypertriglyceridemia with diabetes mellitus.

The side effects of tamoxifen are generally mild, including the effect on lipoprotein metabolism. However, there are few cases of severe tamoxifen induced hypertriglyceridemia. Hypertriglyceridemia is a marked risk factor for acute pancreatitis and approximately 2% to 5% of cases of acute pancreatitis are related to drugs. We report on tamoxifen-induced hypertriglyceridemia and acute pancreatitis in a 40 years old woman with type 2 diabetes mellitus occurred by dexamethasone. She was treated with insulin infusion and fenofibrate, and goserelin acetate was started instead of tamoxifen after discharge from the hospital. Also, probable pathogenic hypotheses about the correlation between tamoxifen and dexamethasone induced type 2 diabetes mellitus on severe acute pancreatitis are provided. Clinicians should take care of risks of severe acute pancreatitis on using tamoxifen, especially for patients with dexamethasone induced diabetes mellitus. These individuals should undergo pre-post tamoxifen lipid screening and careful history taking of drugs, including dexamethasone.

Ginseng Berry Extract Prevents Atherogenesis via Anti-Inflammatory Action by Upregulating Phase II Gene Expression.

Ginseng berry possesses higher ginsenoside content than its root, which has been traditionally used in herbal medicine for many human diseases, including atherosclerosis. We here examined the antiatherogenic effects of the Korean ginseng berry extract (KGBE) and investigated its underlying mechanism of action in vitro and in vivo. Administration of KGBE decreased atherosclerotic lesions, which was inversely correlated with the expression levels of phase II genes to include heme oxygenase-1 (HO-1) and glutamine-cysteine ligase (GCL). Furthermore, KGBE administration suppressed NF-κB-mediated expression of atherogenic inflammatory genes (TNF-α, IL-1ß, iNOS, COX-2, ICAM-1, and VCAM-1), without altering serum cholesterol levels, in ApoE(-/-) mice fed a high fat-diet. Treatment with KGBE increased phase II gene expression and suppressed lipopolysaccharide-induced reactive oxygen species production, NF-κB activation, and inflammatory gene expression in primary macrophages. Importantly, these cellular events were blocked by selective inhibitors of HO-1 and GCL. In addition, these inhibitors reversed the suppressive effect of KGBE on TNF-α-mediated induction of ICAM-1 and VCAM-1, resulting in decreased interaction between endothelial cells and monocytes. These results suggest that KGBE ameliorates atherosclerosis by inhibiting NF-κB-mediated expression of atherogenic genes via upregulation of phase II enzymes and thus has therapeutic or preventive potential for atherosclerosis.

The prognoses of metaplastic breast cancer patients compared to those of triple-negative breast cancer patients.

Metaplastic breast carcinoma (MBC) is a rare, heterogeneous breast cancer characterized by admixture of adenocarcinoma with metaplastic elements, low hormone receptor expression, and poor outcomes. The authors retrospectively reviewed the medical records of 47 MBC patients and 1,346 invasive ductal carcinoma (IDC) patients. Two hundred eighteen of the IDC patients were triple-negative (TN-IDC) for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor-2 (ER-/PR-/HER2-). Patients were surgically treated at the Samsung Medical Center between 2005 and 2009. The MBC patients presented with a larger tumor size, lower lymph node involvement, higher histological and nuclear grades, higher triple negativity (ER-/PR-/HER2-) and higher p53, CK5/6, and EGFR expressions compared with those of the IDC group. However, there were no significant differences in clinicopathological characteristics between MBC and TN-IDC. During the follow-up period (median duration of 30.3 months, range 2.6-56.3 months), seven (14.9%) MBC patients, and 98 (7.1%) IDC patients had disease recurrence. The three-year disease-free survival (DFS) rate was 78.1% in the MBC group and 91.1% in IDC group (P < 0.001). The three-year DFS rate was not significantly different between the MBC and TN-IDC groups (78.1 vs. 84.9%, P = 0.114). However, in patients with lymph node metastasis who underwent adjuvant chemotherapy, the three-year DFS rate was 44.4% in the MBC group and 72.5% in the TN-IDC group (P = 0.025). The authors found that MBC had a poorer clinical outcome than did IDC. In breast cancer patients with nodal metastasis, MBC had a poorer prognosis than did TN-IDC, despite adjuvant chemotherapy.

Large clinical experience of primary angiosarcoma of the breast in a single Korean medical institute.

BACKGROUND: Angiosarcoma of the breast is rare. The purpose of the present study was to evaluate the clinicopathologic characteristics and the clinical outcomes of patients with primary breast angiosarcoma. METHODS: We analyzed the clinicopathologic factors of patients with angiosarcoma of the breast treated between 1997 and 2010 at the Samsung Medical Center. We reviewed the related demographic data, preoperative imaging studies, method of histologic confirmation, tumor size, histologic grades, status of hormonal receptors, treatment modality, and survival data. RESULTS: Nine women with angiosarcoma of the breast were identified. The median age of patients with primary angiosarcoma of the breast at diagnosis was 31 years (range: 19-63 years), and the median tumor size was 9.0 cm (range: 3.5-10.7 cm). Seven patients (77.7%) died within a median follow-up of 46.7 months (range: 3.9-63.3 months), and all deaths were directly attributed to angiosarcoma. Overall, the median time from diagnosis of angiosarcoma to death was 46.8 months (range: 8.21-63.3 months). The 5 year overall survival with angiosarcoma was 42.9%. CONCLUSIONS: Primary angiosarcoma of the breast is a rare malignancy with a poor prognosis, even with complete resection. Chemotherapy and radiation therapy have limited value as treatments to date. Total mastectomy appears to be the most appropriate and beneficial treatment. More aggressive surgical management should be considered, and future clinical research should explore the most appropriate adjuvant therapy in the treatment of angiosarcoma.

Ileal metastasis of breast cancer in a patient with a BRCA2 gene mutation: report of a case.

Breast cancer is the most common malignancy in females. Common sites of metastases include the liver, lung, bone, and brain, while metastases to the extrahepatic digestive system are very rare. This report presents a patient diagnosed with breast carcinoma metastasis in the terminal ileum. The patient underwent breast-conserving surgery on both breasts because of breast cancer at the age of 46 years. Both breast cancers were consistent with stage I invasive ductal carcinomas. Colonoscopy during an investigation for hematochezia revealed a 2-cm ulceration in the terminal ileum 22 months later, and microscopic examination of a biopsy specimen of the ulceration revealed a poorly differentiated mass that was strongly suggestive of metastatic adenocarcinoma with endolymphatic tumor emboli. She underwent hand-assisted laparoscopic ileocecectomy because of ileal metastasis. She had a family history of breast cancer (sister) and colon cancer (brother). She exhibited HER2/neu discordance and carried the BRCA2 gene mutation. Surgeons should remain aware that breast cancer can metastasize to the gastrointestinal tract.

Risk Factors of Unfavorable Outcomes, Major Bleeding, and All-Cause Mortality in Patients with Venous Thromboembolism.

PURPOSE: This study aimed to analyze the clinical outcomes of venous thromboembolism (VTE) patients and identify the risk factors for VTE-related unfavorable outcomes, major bleeding, and 30-day all-cause mortality. MATERIALS AND METHODS: From January 2016 to December 2020, 198 patients with confirmed VTE were enrolled. Potential risk factors for unfavorable outcomes, major bleeding, and all-cause mortality were analyzed. RESULTS: VTE-related unfavorable outcomes developed in 13.1%, while 30-day all-cause mortality was 8.6%. In the multivariate analysis, a pulse ≥110/min and respiratory rate ≥30/min were statistically significant predictors for VTE-related unfavorable outcomes. Diabetes was a significant risk factor for major bleeding. In addition, a history of malignancy, no anticoagulation treatment, and need for mechanical ventilation were significant predictors of all-cause mortality. CONCLUSION: VTE-related mortality and morbidity rates remained high. In cases of tachycardia and tachypnea, early aggressive treatment is needed to prevent unfavorable outcomes. Patients with risk factors should be closely monitored.

Long-term Outcomes of Direct Endoscopic Necrosectomy for Complicated or Symptomatic Walled-Off Necrosis: A Korean Multicenter Study.

Background/Aims: The endoscopic step-up approach is accepted as the preferred treatment for complicated or symptomatic walled-off necrosis (WON). Direct endoscopic necrosectomy (DEN) is an effective therapeutic option, but few reports describe long-term follow-up in this patient population. Thus, we aim to assess the long-term outcomes of DEN following severe necrotizing pancreatitis. Methods: The data of all acute pancreatitis patients who underwent DEN following endoscopic transmural drainage from six referral centers between 2007 and 2017 were retrospectively collected. Results: Sixty patients (76.7% male, mean age 48.3 years) underwent a median of 4 sessions of DEN starting at a median of 45.5 days after the onset of acute pancreatitis. Clinical success was achieved in 51 patients (85%), with a 35% complication rate and a 5% mortality rate. Using multivariate analysis, the risk factor associated with DEN failure or major DEN complications requiring intervention or surgery was an identified bacterial/fungal WON infection (odds ratio, 19.3; 95% confidence interval, 1.5 to 261.7). During the median follow-up period of 27 months, complicated WON recurrence was observed in 5.3% of patients, and long-term complications occurred in 24.6% of patients (four exocrine insufficiency, nine newly developed diabetes mellitus, one recurrent small bowel obstruction, one chylous ascites). Conclusions: Considering that long-term complications are similar to those observed after pancreatectomy, DEN should be performed meticulously while minimizing damage to the viable pancreatic parenchyma with adequate antibiotic escalation.

Pifithrin-α, an inhibitor of p53 transactivation, up-regulates COX-2 expression through an MAPK-dependent pathway.

Cyclooxygenase-2 (COX-2) has been reported to be elevated in many cancers, including breast and colorectal cancers, resulting in accumulation of prostaglandin E2 in the cancer cell environment. In this study, we investigated the effect of pifithrin (PFT)-α, an inhibitor of p53 transactivation, on COX-2 expression in breast and fibrosarcoma cells. Our results showed that COX-2 expression was dose-dependently increased by PFT-α in MDA-MB231 breast cancer cells with mutant p53. In addition, the expression level of COX-2 was also increased by PFT-α in normal fibroblasts as well as in HT1080 fibrosarcoma cells with p53 wild-type cells. To verify the regulatory mechanism of COX-2 in response to PFT-α, we pretreated cells with a mitogen-activated protein kinase (MAPK) kinase (MEK)1/2 inhibitor (UO126) and a phosphoinositide-3 (PI-3K) inhibitor (LY294002). PFT-α-induced COX-2 expression was significantly decreased by UO126 and LY294002 in MDA-MB231 cells. However, the phosphorylation of extracellular signal-regulated kinase (ERK) was increased by PFT-α, but not Akt phosphorylation. Finally, we confirmed the correlation of the MEK and PI-3K pathway and COX-2 expression using the constitutively active (CA)-MEK and myr-Akt adenovirus systems. COX-2 expression was increased by CA-MEK transfection, but not by myr-Akt. Taken together, we have demonstrated that PFT-α-induced COX-2 expression is regulated through a MEK/ERK pathway in MDA-MB231 human breast cancer cells.

Type 2 Autoimmune Pancreatitis (Idiopathic Duct-Centric Pancreatitis) Highlighting Patients Presenting as Clinical Acute Pancreatitis: A Single-Center Experience.

Background/Aims: Type 2 autoimmune pancreatitis (AIP) has been considered extremely rare in East Asia. This study aimed to clarify the prevalence, clinical characteristics and radiological findings of type 2 AIP highlighting patients presenting as acute pancreatitis in a single center. Methods: Type 2 AIP patients were classified according to International Consensus Diagnostic Criteria. Radiological findings were compared between type 2 AIP presenting as acute pancreatitis and gallstone pancreatitis. Results: Among 244 patients with AIP, 27 (11.1%) had type 2 AIP (definite, 15 [55.5%] and probable 12 [44.5%]). The median age of patients with type 2 AIP was 29 years (interquartile range, 20 to 39 years). Acute pancreatitis was the most common initial presentation (n=17, 63%) while obstructive jaundice was present in only one patient. Ulcerative colitis (UC) was associated with type 2 AIP in 44.4% (12/27) of patients. Radiological pancreatic imaging such as delayed enhancement of diffusely enlarged pancreas, homogeneous enhancement of focal enlargement/mass, absent/minimal peripancreatic fat infiltration or fluid collection, and multifocal main pancreatic duct narrowings were helpful for differentiating type 2 AIP from gallstone pancreatitis. During follow-up (median, 32.3 months), two patients (2/25, 8%) experienced relapse. Conclusions: In South Korea, type 2 AIP is not as rare as previously thought. Overall, the clinical profile of type 2 AIP was similar to that of Western countries. Type 2 AIP should be considered in young UC patients with acute pancreatitis of uncertain etiology.

Relapse rate and predictors of relapse in a large single center cohort of type 1 autoimmune pancreatitis: long-term follow-up results after steroid therapy with short-duration maintenance treatment.

BACKGROUND: Type 1 autoimmune pancreatitis (AIP), as a pancreatic manifestation of IgG4-related disease, shows a favorable prognosis in the short term. However, disease relapse is common in long-term follow-up, despite a successful initial treatment response. This study aimed to identify the predictors of relapse and long-term outcomes in patients with type 1 AIP. METHODS: Patients with more than 2 years of follow-up who met the International Consensus Diagnostic Criteria for type 1 AIP were included. Patients who had undergone pancreatic operations associated with AIP or who lacked sufficient clinical data were excluded. RESULTS: All 138 patients achieved clinical remission with initial steroid therapy, and 66 (47.8%) experienced relapse during a median 60 (range 24-197) months follow-up. Among the relapsed patients, about 74% (49/66) relapsed within 3 years. About 60% (82/138) had other organ involvement (OOI), most commonly in the proximal bile duct (26.8%). At first diagnosis, OOI, and especially OOI of the proximal bile duct, was a significant independent predictor of relapse (hazard ratio 2.65; 95% confidence interval 1.44-4.89; p = 0.002), according to multivariate analysis. During the follow-up period, 16 (11.6%) patients experienced endocrine/exocrine dysfunction and 32 (23.2%) patients developed de novo pancreatic calcifications/stones. No pancreatic cancer occurred in any patients. CONCLUSIONS: Type 1 AIP has common relapses, and patients with OOI, especially OOI of the proximal bile duct, appear to be at increased risk for relapse. Long-term sequelae, including pancreatic insufficiency and pancreatic calcifications/stones, are common in patients with relapse. To reduce the relapse, longer maintenance treatment may be needed especially for patients at high risk for relapse.