Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment.

Background and aimsAutoimmune pancreatitis (AIP) is an immune-mediated disease of the pancreas with distinct pathophysiology and manifestations. Our aims were to characterize type 1 AIP in a large pan-European cohort and study the effectiveness of current treatment regimens.MethodsWe retrospectively analyzed adults diagnosed since 2005 with type 1 or not-otherwise-specified AIP in 42 European university hospitals. Type 1 AIP was uniformly diagnosed using specific diagnostic criteria. Patients with type 2 AIP and those who had undergone pancreatic surgery were excluded. The primary endpoint was complete remission, defined as the absence of clinical symptoms and resolution of the index radiological pancreatic abnormalities attributed to AIP.ResultsWe included 735 individuals with AIP (69% male; median age 57 years; 85% White). Steroid treatment was started in 634 patients, of whom 9 (1%) were lost to follow-up. The remaining 625 had a 79% (496/625) complete, 18% (111/625) partial, and 97% (607/625) cumulative remission rate, while 3% (18/625) did not achieve remission. No treatment was given in 95 patients, who had a 61% complete (58/95), 19% partial (18/95), and 80% cumulative (76/95) spontaneous remission rate. Higher (≥0.4 mg/kg/day) corticosteroid doses were no more effective than lower ( 2 weeks (OR 0.908; 95%CI 0.818-1.009). Elevated IgG4 levels were independently associated with a decreased chance of complete remission (OR 0.639; 95%CI 0.427-0.955). Relapse occurred in 30% of patients. Relapses within 6 months of remission induction were independent of the steroid tapering duration, induction treatment duration, and total cumulative dose.ConclusionPatients with type 1 AIP and elevated IgG4 level may need closer monitoring. For remission induction, a starting dose of 0.4 mg/kg/day for 2 weeks followed by a short taper period seems effective. This study provides no evidence to support more aggressive regimens

Mutation analysis of PRSS1, SPINK1 and CFTR gene in patients with alcoholic and idiopathic chronic pancreatitis: A single center study

Background/Aims: A relation between some genetic mutations and chronic pancreatitis (CP) has been reported. However, the relation of genetic mutation to alcoholic CP (ACP) and idiopathic CP (ICP) still remains controversial. In this study, we investigated the prevalence of protease serine 1 (PRSS1), serine protease inhibitor, Kazal type 1 (SPINK1) SPINK1 and cystic fibrosis transmembrane conductance regulator (CFTR) mutations in ACP and ICP patients in Turkey

The Role of EUS and EUS-FNA in the Management of Pancreatic Masses: Five-Year Experience

Background/Aims: The efficacy of endoscopic ultrasound with fine-needle aspiration (EUS-FNA) in the diagnosis and staging of pancreatic malignancy is quite well established. The aim of this study is to describe a single-centre's experience. Methodology: Data were collected retrospectively on all patients with solid pancreatic masses undergoing EUS-FNA from January 2006 to March 2011. In tumor cases, TNM staging using EUS was compared with the results of histopathological staging. Results: EUS-FNA of pancreatic lesions was performed in 125 patients. Of these patients, data of 75 were available (68% men, mean age 59.97 +/- 11.12 (SD) years); 58 (77%) of the lesions were ductal adenocarcinoma, 11 (14.5%) were chronic pancreatitis, 4 (%5) were intraductal papillary mucinous carcinoma (IPMN) and 2 (%3) were insulinoma. Diagnostic yield of the EUS-FNA procedure was 74.7% (56/75). Specimens from six patients were inadequate. In multivariate analysis, lesion diameter below 2cm was an independent predictor for the inadequacy of material (p=0.04). Conclusions: In patients with pancreatic mass with suspected malignancy, EUS-FNA provided a diagnosis with accuracy rate of 75%. Inadequate material with EUS-FNA was significantly more frequent in lesions below 2cm

Endosonography-assisted transmural endoscopic drainage of pancreatic pseudocysts: A single center experience

Background/aims: Management of pancreatic pseudocysts can be challenging. Endosonography-guided drainage of the pseudocysts is an important treatment modality. In this study, we evaluated the results of endosonography-guided transmural endoscopic drainage of these lesions. Materials and Methods: We performed drainage of the pancreatic pseudocysts through the stomach or duodenum using a linear endosonography device. The procedure steps were as follows: Determination of the best location for needle insertion, puncture of the cyst, guide insertion, creation of a window between the cyst and stomach lumen using a cystotome, and finally insertion of a double pigtail catheter. Results: The procedure was applied to 12 patients (8 males, 4 females, mean age: 51 +/- 15.6 years), with success achieved in 10 patients (83%), defined as complete disappearance of the cyst. The mean cyst diameter was 9 cm (range: 6-12 cm). There was only one complication (8%), as an intraabdominal abscess with an uncomplicated course after surgical drainage. Conclusions: Endosonography-guided drainage is an effective and safe method for the management of pancreatic pseudocysts

Familial chylomicronemia syndrome related chronic pancreatitis: a single-center study

BACKGROUND: Hypertriglyceridemia induces acute recurrent pancreatitis, but its role in the etiology of chronic pancreatitis (CP) is controversial. This study aimed to evaluate the clinical, laboratory and radiological findings of 7 patients with CP due to type 1 hyperlipidemia compared to CP patients with other or undefined etiological factors

Predictors of endoscopic recurrence in resected patients with Crohn's disease in a long-term follow-up cohort: History of multiple previous resections and residual synchronous disease in the remnant intestine

Aims: This study aimed to determine the predictors of endoscopic recurrence in a cohort of patients with Crohn's disease (CD) with prior intestinal resections