CASE REPORT
Autoimmune Pancreatitis in Children: Insights Into the Diagnostic Challenge
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Yoh Zen, yTassos Grammatikopoulos, and yNedim Hadzic
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utoimmune pancreatitis (AIP) is increasingly recognised in adults (1). Clinical features include obstructive jaundice, radiological signs of a diffuse pancreatic enlargement and irregular narrowing of the pancreatic duct, increased serum levels of immunoglobulin G4 (IgG4), variable presence of autoantibodies, and clinical response to steroids (1). An international consensus statement was published suggesting 2 distinct variants: type 1 AIP, seen in approximately 85% of adult patients and characterized by elevated serum IgG4 concentrations and association with IgG4related disease at extrapancreatic sites (eg, sialadenitis, retroperitoneal fibrosis), and type 2 AIP, in which the serological markers are usually absent with no systemic manifestations except for possible association with inflammatory bowel disease (2). Histologically, type 1 AIP shows massive infiltration by IgG4þ plasma cells, whereas the type 2 AIP is characterized by neutrophilic infiltration with granulocytic epithelial lesions (GELs) and few, if any, IgG4þ plasma cells (1–4). We describe our experience in diagnosing AIP in 2 children and discuss how to improve diagnostic approach to this condition.
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PATIENT 1 An 11-year-old boy presented with epigastric pain, jaundice, and weight loss. Blood tests showed bilirubin 225 mmol/L (normal range 3–20), g-glutamyl transpeptidase 120 IU/L (1–55), alkaline phosphatase 597 IU/L (120–488), aspartate aminotransferase 78 IU/L (10–50), and amylase 33 IU/L (
Autoimmune Pancreatitis in Children: Insights Into the Diagnostic Challenge
A
Yoh Zen, yTassos Grammatikopoulos, and yNedim Hadzic
A
utoimmune pancreatitis (AIP) is increasingly recognised in adults (1). Clinical features include obstructive jaundice, radiological signs of a diffuse pancreatic enlargement and irregular narrowing of the pancreatic duct, increased serum levels of immunoglobulin G4 (IgG4), variable presence of autoantibodies, and clinical response to steroids (1). An international consensus statement was published suggesting 2 distinct variants: type 1 AIP, seen in approximately 85% of adult patients and characterized by elevated serum IgG4 concentrations and association with IgG4related disease at extrapancreatic sites (eg, sialadenitis, retroperitoneal fibrosis), and type 2 AIP, in which the serological markers are usually absent with no systemic manifestations except for possible association with inflammatory bowel disease (2). Histologically, type 1 AIP shows massive infiltration by IgG4þ plasma cells, whereas the type 2 AIP is characterized by neutrophilic infiltration with granulocytic epithelial lesions (GELs) and few, if any, IgG4þ plasma cells (1–4). We describe our experience in diagnosing AIP in 2 children and discuss how to improve diagnostic approach to this condition.
B
C
PATIENT 1 An 11-year-old boy presented with epigastric pain, jaundice, and weight loss. Blood tests showed bilirubin 225 mmol/L (normal range 3–20), g-glutamyl transpeptidase 120 IU/L (1–55), alkaline phosphatase 597 IU/L (120–488), aspartate aminotransferase 78 IU/L (10–50), and amylase 33 IU/L (
