Abstract
Immunoglobulin subclass 4 related disease (IgG4-RD) is an increasingly recognised autoimmune disease with the potential of affecting various organs. It has a predilection for certain anatomical hotspots and the pancreatobiliary tract is the the most common area involved. Due to the relative novelty of IgG4-RD, the understanding of the disease process continues to involve. Recent European guidelines on IgG4-RD have been published by a working group collaboration between the United European Gastroenterology and Swedish Society of Gastroenterology. In our commentary, we aim to extract the key practical points with an emphasis on diagnosis and management of IgG4-RD with specific focus on the pancreatobiliary tract.