Autoimmune pancreatitis not otherwise specified (NOS): Clinical features and outcomes of the forgotten type.

BACKGROUND: Autoimmune pancreatitis (AIP) is a well-recognized fibroinflammatory disease of the pancreas. Despite the significant number of studies published on AIP type 1 and 2, no studies have been focused on AIP type not otherwise specified (NOS) and therefore very little is known about clinical features and long-term outcomes of these patients. The aim of this study was to investigate clinical and radiological features of AIP type NOS-patients. METHODS: Patients classified as AIP type NOS at clinical onset included in our database prospectively maintained since 1995 were evaluated. Epidemiological, clinical data were collected and analyzed. RESULTS: Forty-six patients were included in the study. The clinical onset was mainly characterized by weight loss, jaundice and acute pancreatitis. Eight patients (17.4%) were reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis. Seven patients (15.2%) experienced relapse after steroid treatment but only one (2.2%) needed immunosuppressive drugs because of recurrent relapses. CONCLUSIONS: AIP type NOS shares clinical features similar to AIP type 2 and a relevant proportion of patients was reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis. The risk of relapse is low but not irrelevant.

Secondary analysis of large databases for hepatology research.

Secondary analysis of large datasets involves the utilization of existing data that has typically been collected for other purposes to advance scientific knowledge. This is an established methodology applied in health services research with the unique advantage of efficiently identifying relationships between predictor and outcome variables but which has been underutilized for hepatology research. Our review of 1431 abstracts published in the 2013 European Association for the Study of Liver (EASL) abstract book showed that less than 0.5% of published abstracts utilized secondary analysis of large database methodologies. This review paper describes existing large datasets that can be exploited for secondary analyses in liver disease research. It also suggests potential questions that could be addressed using these data warehouses and highlights the strengths and limitations of each dataset as described by authors that have previously used them. The overall goal is to bring these datasets to the attention of readers and ultimately encourage the consideration of secondary analysis of large database methodologies for the advancement of hepatology.

Rituximab as Maintenance Therapy in Type 1 Autoimmune Pancreatitis: An Italian Experience.

OBJECTIVE: Rituximab (RTX) has been proposed for the induction of remission and maintenance therapy in relapsing type 1 autoimmune pancreatitis (AIP). The aim of the study was to describe the use of RTX as maintenance therapy for patients with type 1 AIP. METHODS: Patients with type 1 AIP based on the International Consensus Diagnostic Criteria and treated with RTX were selected from our database. Two doses of RTX (1000 mg each) were administered 15 days apart and repeated after 6 months. RESULTS: Eighteen patients were treated with RTX as maintenance therapy. Of these, the involvement of other organs was observed in 16 patients (89%). Eight of the 18 patients (44%) relapsed during follow-up. Median time to relapse after the last infusion was 30 months (range, 12-35 months). No disease relapse was observed in the first year after the last infusion. Probability of disease relapse was 80% between 1 and 3 years from initial treatment. No adverse effects were observed. CONCLUSIONS: Rituximab seems be safe and effective for maintenance therapy of type 1 AIP during the first year after completing RTX infusion. However, the probability of disease relapse is high within 1 and 3 years from the last infusion.

Updates in the field of autoimmune pancreatitis: a clinical guide.

INTRODUCTION: Autoimmune Pancreatitis (AIP) is a peculiar form of pancreatitis different from all other type of pancreatitis ('one like no one pancreatitis') and characterized by a dramatic response to steroids. AIP can be classified histologically in type 1, included in a larger group of diseases named IgG4-related disease, and type 2. At imaging, AIP may involve all (diffuse form) or only a part (focal form) of the pancreatic gland. Areas covered: In this article, the clinical approach to diagnosis and different therapeutic strategies of this complicated disease are reviewed. Literature search was undertaken using PubMed database entering autoimmune pancreatitis [title] or IgG4-related diseases [title], and selecting the more relevant papers for diagnosis and therapy of AIP in clinical practice. We focus on diagnosis of AIP in focal and diffuse form, and how to achieve diagnosis for type 1 and type 2 AIP. We finally analyzed the different strategies proposed in induction of remission and maintenance therapy (long-term low-dose steroids, immunosuppressants or biologics). Expert commentary: The main issue in clinical practice is how to achieve the diagnosis. A second key point is how to prevent the disease relapse.

Nutritional support and therapy in pancreatic surgery: A position paper of the International Study Group on Pancreatic Surgery (ISGPS).

BACKGROUND: The optimal nutritional therapy in the field of pancreatic surgery is still debated. METHODS: An international panel of recognized pancreatic surgeons and pancreatologists decided that the topic of nutritional support was of importance in pancreatic surgery. Thus, they reviewed the best contemporary literature and worked to develop a position paper to provide evidence supporting the integration of appropriate nutritional support into the overall management of patients undergoing pancreatic resection. Strength of recommendation and quality of evidence were based on the approach of the grading of recommendations assessment, development and evaluation Working Group. RESULTS: The measurement of nutritional status should be part of routine preoperative assessment because malnutrition is a recognized risk factor for surgery-related complications. In addition to patient's weight loss and body mass index, measurement of sarcopenia and sarcopenic obesity should be considered in the preoperative evaluation because they are strong predictors of poor short-term and long-term outcomes. The available data do not show any definitive nutritional advantages for one specific type of gastrointestinal reconstruction technique after pancreatoduodenectomy over the others. Postoperative early resumption of oral intake is safe and should be encouraged within enhanced recovery protocols, but in the case of severe postoperative complications or poor tolerance of oral food after the operation, supplementary artificial nutrition should be started at once. At present, there is not enough evidence to show the benefit of avoiding oral intake in clinically stable patients who are complicated by a clinically irrelevant postoperative pancreatic fistula (a so-called biochemical leak), while special caution should be given to feeding patients with clinically relevant postoperative pancreatic fistula orally. When an artificial nutritional support is needed, enteral nutrition is preferred whenever possible over parenteral nutrition. After the operation, regardless of the type of pancreatic resection or technique of reconstruction, patients should be monitored carefully to assess for the presence of endocrine and exocrine pancreatic insufficiency. Although fecal elastase-1 is the most readily available clinical test for detection of pancreatic exocrine insufficiency, its sensitivity and specificity are low. Pancreatic enzyme replacement therapy should be initiated routinely after pancreatoduodenectomy and in patients with locally advanced disease and continued for at least 6 months after surgery, because untreated pancreatic exocrine insufficiency may result in severe nutritional derangement. CONCLUSION: The importance of this position paper is the consensus reached on the topic. Concentrating on nutritional support and therapy is of utmost value in pancreatic surgery for both short- and long-term outcomes.