IgG4-related systemic sclerosing disease: a diagnosis to be considered

IgG4-related systemic sclerosing disease is a chronic autoimmune disorder with diverse clinical presentation. We report a 56-year-old patient who has presented with obstructive jaundice, bulky pancreas and mesenteric lymph node mass. He was later diagnosed to have IgG4-sclerosing disease involving pancreas, small bowel mesentery, common bile duct, liver, gallbladder and submandibular salivary glands and has required multiple surgical interventions. IgG4-related systemic sclerosing disease appears to be more commonly encountered than it was once thought and needs considered in patients presenting with pancreatitis of no obvious underlying cause. Although the disease is primarily managed by medical therapy, there is a role for surgical intervention in order to obtain tissue for diagnosis, exclude other possible neoplastic aetiology and to manage disease-related complications.