Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood: Consensus From INSPPIRE

Abstract

Objectives: Autoimmune pancreatitis (AIP) represents a complex immune-mediated pancreas disorder. Pediatric AIP (P-AIP) is rare. We have recently summarized the characteristic features of P-AIP. We now aim to develop recommendation statements to standardize the diagnostic and therapeutic approach to P-AIP and facilitate future research in the field.

Methods: A panel of pediatric gastroenterologists participating in the International Study Group of Pediatric Pancreatitis: In search for a cuRE was formed to discuss and then vote on 15 recommendation statements. A consensus of at least 80% was obtained following 3 voting rounds and revision of the statements.

Results: We have now generated 15 statements to help standardize the approach to diagnosis and management of P-AIP.

Conclusions: The first P-AIP recommendation statements developed by the International Study Group of Pediatric Pancreatitis: In search for a cuRE group are intended to bring standardization to the diagnosis and treatment of this rare childhood disorder. These statements may help guide a uniform approach to patient care and facilitate future research studies.

Figure 1. Imaging characteristics in pediatric autoimmune pancreatitis patients

A. Magnetic resonance cholangiopancreatography (MRCP) T1-weighted transversal section evidencing a pathologic hyperintense capsule-like rim at the pancreas periphery. B. MRCP T2-weighted three-dimensional (3D) cholangiogram reconstruction showing a dilated common bile duct (CBD) that is obstructed in its proximal intrapancreatic part (arrow). The main pancreatic duct (MPD) also narrows in the pancreas head. C. Transabdominal ultrasound showing a bulky hypoechoic heterogeneous pancreatic head (arrows). D. MRCP T2-weighted transversal section showing an enlarged pancreas head (arrows) with a hypointense mass lesion (dotted area) inside.