Autoimmune Pancreatitis and IgG4 Related Disease in Three Children.
We report 3 children who presented with fever and abdominal pain, deranged liver function tests, and on abdominal ultrasound were found to have an enlarged pancreas, substantial abdominal lymphadenopathy, and extrahepatic biliary duct dilatation. After ruling out malignancy, probable immunoglobulin G4-related disease (IgG4RD) associated with autoimmune pancreatitis was considered. This condition was first described in the adults and often mimics pancreatic cancer. It can involve multiple organs, either synchronously or metachronously, and is rarely reported in children. The disorder mostly responds to corticosteroid therapy and other immune suppression. We highlight the difficulty in diagnosing autoimmune pancreatitis/IgG4-related disease in children and illustrate the difference between pediatric and adult presentation.
Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Australia.
Rishi
R
Bolia
Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Australia.
Sze Yee
SY
Chong
Department of Radiology, Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Childrens Research Institute, Parkville, Melbourne, Australia.
Lee
L
Coleman
Department of Anatomical Pathology, Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia.
Duncan
D
MacGregor
Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Childrens Research Institute, Parkville, Melbourne, Australia.
Winita
W
Hardikar
Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Childrens Research Institute, Parkville, Melbourne, Australia.
Mark R
MR
Oliver