Two clinical cases of IgG4-associated pancreatitis in children

Abstract

Autoimmune pancreatitis is a specific peculiar and rather new form of chronic pancreatitis (ChP) that has been intensively studied by pancreatology specialists of the whole world for the last years. Autoimmune pancreatitis does not have any individual specific clinical features. This condition was described for the first time in adults and it often mimics pancreas cancer. Autoimmune pancreatitis can involve some organs, synchronously or metachronously, and is rarely identified in children. Autoimmune pancreatitis is a systemic fiber-inflammatory condition with poorly understood pathophysiological mechanism, affecting different parts of the body. In case of the treatment absence the disease can lead to fibrosis and irreversible impairment of the organs. However, the combination of the findings of serologic, histologic, and instrumental methods of investigation characteristic for аutoimmune pancreatitis allows to identify its presence. IgG4-associated disorder has been mainly described in adults, therefore pediatricians usually do not know much about it. We underline the complexity of the diagnosis of autoimmune pancreatitis / IgG4-associated disorder, in children. The article presents two clinical cases of children diagnosed with autoimmune pancreatitis. Both children had the symptoms of abdominal pain and/or mechanical jaundice combined with focal pancreas enlargement, roughness of the basic pancreas duct and distal narrowing. According to our data, the diagnosis of autoimmune pancreatitis in children can be made on the basis of the combination of specific clinical symptoms at admission and various results of imaging. Both timely made diagnosis and administered treatment provide a favorable prognosis for further course of the disease and complication development.