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.

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

Introduction

Achalasia is a primary motor disorder of the oesophagus characterised by absence of peristalsis and insufficient lower oesophageal sphincter relaxation. With new advances and developments in achalasia management, there is an increasing demand for comprehensive evidence-based guidelines to assist clinicians in achalasia patient care.

Methods
These guidelines were established in accordance with the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument. A systematic review of the literature was performed and the certainty in the evidence was assessed using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology. Recommendations were voted upon using a nominal group technique.

Results
These guidelines specifically focus on the definition of achalasia, treatment aims, diagnostic tests, medical, endoscopic and surgical therapy, management of treatment failure, follow-up and oesophageal cancer risk.

Conclusion
These multidisciplinary UEG and ESNM guidelines provide a comprehensive evidence-based framework with recommendations on the diagnosis, treatment and follow-up of adult achalasia patients.

Keywords: Dysphagia; oesophagus; manometry; myotomy; motility; esophagus