The overall objective of these guidelines is to provide evidence-based recommendations for the diagnosis and management of immunoglobulin G4 (IgG4)-related digestive disease in adults and children. IgG4-related digestive disease can be diagnosed only with a comprehensive work-up that includes histology, organ morphology atimaging, serology, search for other organ involvement, and response to glucocorticoid treatment. Indications for treatment are symptomatic patients with obstructive jaundice, abdominal pain, posterior pancreatic pain, and involvement of extra-pancreatic digestive organs, including IgG4-related cholangitis. Treatment with glucocorticoids should be weight-based and initiated at a dose of 0.6–0.8 mg/kg body weight/day orally (typical starting dose 30-40 mg/day prednisone equivalent) for 1 month to induce remission and then be tapered within two additional months. Response to initial treatment should be assessed at week 2–4 with clinical, biochemical and morphological markers. Maintenance treatment with glucocorticoids should be considered in multi-organ disease or history of relapse. If there is no change in disease activity and burden within 3 months, the diagnosis should be reconsidered. If the disease relapsed during the 3 months of treatment, immunosuppressive drugs should be added.

Keywords: IgG4-related; digestive; disease; glucocorticoids; other organ involvement; biomarkers; autoimmune pancreatitis type 1; immune-related cholangitis; cancer; diabetes mellitus

Both acute and chronic pancreatitis are frequent diseases of the pancreas, which, despite being of benign nature, are related to a significant risk of malnutrition and may require nutritional support. Acute necrotizing pancreatitis is encountered in 20% of patients with acute pancreatitis, is associated with increased morbidity and mortality, and may require artificial nutrition by enteral or parenteral route, as well as additional endoscopic, radiological or surgical interventions. Chronic pancreatitis represents a chronic inflammation of the pancreatic gland with development of fibrosis. Abdominal pain leading to decreased oral intake, as well as exocrine and endocrine failure are frequent complications of the disease. All of the above represent risk factors related to malnutrition. Therefore, patients with chronic pancreatitis should be considered at risk, screened and supplemented accordingly. Moreover, osteoporosis and increased facture risk should be acknowledged in patients with chronic pancreatitis, and preventive measures should be considered.

Keywords: Acute pancreatitis, Chronic pancreatitis, Pancreatic diseases, Nutrition, Nutritional support, Medical Nutrition

An interdisciplinary group of European experts summarizes the value of gastrointestinal ultrasound (GIUS) in the management of three time-critical causes of acute abdomen: bowel obstruction, gastrointestinal perforation and acute ischemic bowel disease. Based on an extensive literature review, statements for a targeted diagnostic strategy in these intestinal emergencies are presented. GIUS is best established in case of small bowel obstruction. Metanalyses and prospective studies showed a sensitivity and specificity comparable to that of computed tomography (CT) and superior to plain X-ray. GIUS may save time and radiation exposure and has the advantage of displaying bowel function directly. Gastrointestinal perforation is more challenging for less experienced investigators. Although GIUS in experienced hands has a relatively high sensitivity to establish a correct diagnosis, CT is the most sensitive method in this situation. The spectrum of intestinal ischemia ranges from self-limited ischemic colitis to fatal intestinal infarction. In acute arterial mesenteric ischemia, GIUS may provide information, but prompt CT angiography is the gold standard. On the other end of the spectrum, ischemic colitis shows typical ultrasound features that allow correct diagnosis. GIUS here has a diagnostic performance similar to CT and helps to differentiate mild from severe ischemic colitis.

Keywords: ultrasound - ischemic bowel disease - gastrointestinal tract - bowel obstruction - perforation

Malignant disease

1 ESGE recommends placement of partially or fully covered self-expandable metal stents (SEMSs) for palliation of malignant dysphagia over laser therapy, photodynamic therapy, and esophageal bypass.

Strong recommendation, high quality evidence.

2 ESGE recommends brachytherapy as a valid alternative, alone or in addition to stenting, in esophageal cancer patients with malignant dysphagia and expected longer life expectancy.

Strong recommendation, high quality evidence.

3 ESGE recommends esophageal SEMS placement for sealing malignant tracheoesophageal or bronchoesophageal fistulas.

Strong recommendation, low quality evidence.

4 ESGE does not recommend SEMS placement as a bridge to surgery or before preoperative chemoradiotherapy because it is associated with a high incidence of adverse events. Other options such as feeding tube placement are preferable.

Strong recommendation, low quality evidence.

Benign disease

5 ESGE recommends against the use of SEMSs as first-line therapy for the management of benign esophageal strictures because of the potential for adverse events, the availability of alternative therapies, and their cost.

Strong recommendation, low quality evidence.

6 ESGE suggests consideration of temporary placement of self-expandable stents for refractory benign esophageal strictures.

Weak recommendation, moderate quality evidence.

7 ESGE suggests that fully covered SEMSs be preferred over partially covered SEMSs for the treatment of refractory benign esophageal strictures because of their very low risk of embedment and ease of removability.

Weak recommendation, low quality evidence.

8 ESGE recommends the stent-in-stent technique to remove partially covered SEMSs that are embedded in the esophageal wall.

Strong recommendation, low quality evidence.

9 ESGE recommends that temporary stent placement can be considered for the treatment of leaks, fistulas, and perforations. No specific type of stent can be recommended, and the duration of stenting should be individualized.

Strong recommendation, low quality of evidence.

10 ESGE recommends considering placement of a fully covered large-diameter SEMS for the treatment of esophageal variceal bleeding refractory to medical, endoscopic, and/or radiological therapy, or as initial therapy for patients with massive bleeding.

Strong recommendation, moderate quality evidence.