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 at imaging, 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.

Objectives

No quantitative method has been implemented routinely in clinical practice to assess the oesophago-gastric junction (OGJ). Using impedance planimetry a functional lumen imaging probe (FLIP) was constructed to measure eight cross-sectional areas (CSA) at 4 mm intervals inside a saline-filled bag. To validate the FLIP technique for profiling the OGJ, polymethylmethacrylate (Perspex) cylinders with different CSAs were measured ten times by the FLIP to assess reproducibility and accuracy. A geometric sphincter phantom was constructed and its geometry was measured with a 360 degrees radial ultrasound (US) mini-probe pulled through it at a rate of 1 mm s(-1). The measurements were compared with FLIP measurements. Safety and technique reproducibility were tested on a volunteer. Reproducibility and accuracy between the ten samples were good. The probe performed well with and without a balloon mounted on it except for the smallest CSA (38.5 mm(2)) where there was a difference of 22% from the actual value at one CSA measurement point. The FLIP imaged the phantom geometry as well as the radial scanning US mini-probe. Pilot studies on a volunteer showed that the probe could be placed in the OGJ and the balloon distensions revealed the geometry of the sphincter at various levels of distension. The technique may be useful in accessing the role of the OGJ in diseases such as gastroesophageal reflux disease (GORD) and achalasia and their treatments with surgical and endoscopic therapies.

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