Autoimmune pancreatitis (AIP) is an uncommon cause of pancreatitis that favorably responds to steroid therapy. Its true epidemiology remains unclear because only few cases of pediatric AIP have been reported. According to the adult criteria, AIP can be classified into 2 types: Type 1 or AIP without granulocyte epithelial lesions (GELs), and type 2 or AIP with GEL. Weexperienced two cases of pediatric AIP with unusually early age onset compared to previously reported data. With limited

more »

... , our reported cases have a similar presentation, laboratory findings, radiographic findings, and positive response to steroid therapy compared to the previously reported cases. The main difference consisted in an early onset of disease (3-4 years of age) compared to the average age of onset of 11.9 years. A review of the current available reports demonstrated that more than 70 % of the cases of pediatric AIP could not be conclusively classified into the two types of AIP, raising the concern for a possibility of different pathophysiology between pediatric and adult AIP and the need for a pediatric-specific diagnostic criteria and treatment guideline for AIP.