Varied Presentations and Comorbidities in Pediatric Autoimmune Pancreatitis

Abstract

Autoimmune pancreatitis (AIP) is a chronic inflammatory condition rarely reported in children. In 2018, to standardize the approach to AIP, INternational Study Group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) defined AIP, outlined the clinical course, and developed diagnostic and therapeutic recommendations. We performed a retrospective review of cases at our institution from January 1, 2016, to June 1, 2019, and compared their presentations with the INSPPIRE guidelines. Our patients showed variable laboratory, radiographic, and histologic findings, highlighting the difficulty in diagnosing AIP. Histologic samples were obtained in our patients due to diagnostic uncertainty, which ultimately confirmed the diagnosis. One patient was diagnosed with autoimmune hepatitis coexistent with AIP, which has not been previously described in the pediatric population. Exocrine and endocrine complications of AIP were also noted. In all cases, symptoms improved following treatment, and decompression of the common bile duct was seen on repeat imaging.

Autoimmune pancreatitis (AIP) is a chronic inflammatory condition rarely reported in children. Cases of AIP at a single academic center were reviewed from January 01, 2016, to June 01, 2019. The aim of this report is to describe differences in presentation and diagnostic considerations of pediatric AIP while illustrating comorbid conditions that can occur.