Abstract
Autoimmune pancreatitis is frequently associated with elevated serum and tissue IgG4 levels in the adult population, but there are few reports of pediatric autoimmune pancreatitis, and even fewer reports of IgG4 related systemic disease in a pediatric population. The standard of care treatment in adults is systemic corticosteroids with resolution of symptoms in most cases; however, multiple courses of corticosteroids are occasionally required and some patients require long term corticosteroids. In these instances, steroid sparing disease modify treatments are in demand. We describe a 13-year-old girl with IgG4 related systemic disease who presented with chronic recurrent autoimmune pancreatitis resulting in surgical intervention for obstructive hyperbilirubinemia and chronic corticosteroid treatment. In addition, she developed fibrosing medianstinitis as part of her IgG4 related systemic disease. She was eventually successfully treated with mycophenolate mofetil allowing for discontinuation of corticosteroids. This is the first reported use of mycophenolate mofetil for IgG4 related pancreatitis. Although autoimmune pancreatitis as part of IgG4 related systemic disease is rarely reported in pediatrics, autoimmune pancreatitis is also characterized as idiopathic fibrosing pancreatitis. All pediatric autoimmune pancreatitis cases reported in the world medical literature were identified via a PUBMED search and are reviewed herein. Twelve reports of pediatric autoimmune pancreatitis were identified, most of which were treated with corticosteroids or surgical approaches. Most case reports failed to report IgG4 levels, so it remains unclear how commonly IgG4 related autoimmune pancreatitis occurs during childhood. Increased evaluation of IgG4 levels in patients with autoimmune pancreatitis may shed further light on the association of IgG4 with pancreatitis and the underlying pathophysiology.
Highlights
The concept of immune mediated pancreatitis was first suggested by Sarles et al in 1961 after observing massive lymphoplasmocytic infiltrates and fibrosis on pancreatic histology in patients with pancreatitis of unknown etiology [1]
The patient presented in this report is diagnosable with Autoimmune pancreatitis (AIP) by both Japanese Pancreas Society (JPS) and HISORt diagnostic criteria [3,4,5]
She had an elevated IgG4 with evidence of extrapancreatic manifestations, consistent with IgG4 related systemic disease (ISD), even though IgG4 staining was not done (Table 1 and 2)
Summary
The concept of immune mediated pancreatitis was first suggested by Sarles et al in 1961 after observing massive lymphoplasmocytic infiltrates and fibrosis on pancreatic histology in patients with pancreatitis of unknown etiology [1]. There is one report of a pediatric patient with elevated IgG4 levels; she did not have imaging changes or histology available to make a more definitive diagnosis of AIP [14].
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
