Varied Presentations and Comorbidities in Pediatric Autoimmune Pancreatitis

Area of Science:

• Pediatric Gastroenterology
• Autoimmune Diseases
• Pancreatology

Background:

• Autoimmune pancreatitis (AIP) is a rare chronic inflammatory condition in children.
• The INternational Study Group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) established guidelines in 2018 for diagnosis and management.
• Pediatric AIP cases often present with variable clinical, laboratory, and imaging findings, complicating diagnosis.

Purpose of the Study:

• To retrospectively review pediatric AIP cases at an institution.
• To compare patient presentations with the INSPPIRE guidelines.
• To highlight diagnostic challenges and clinical outcomes in pediatric AIP.

Main Methods:

• Retrospective case review from January 1, 2016, to June 1, 2019.
• Comparison of patient data with INSPPIRE diagnostic and therapeutic recommendations.
• Analysis of laboratory, radiographic, and histologic findings.

Main Results:

• Diagnostic uncertainty necessitated histologic confirmation in several cases.
• One patient presented with coexistent autoimmune hepatitis and AIP, a novel pediatric finding.
• Exocrine and endocrine complications were observed.
• All patients showed symptom improvement post-treatment with evidence of common bile duct decompression on imaging.

Conclusions:

• The INSPPIRE guidelines provide a framework for managing pediatric AIP, though diagnostic variability persists.
• Histologic confirmation is crucial in ambiguous cases.
• AIP can present with coexisting autoimmune conditions and lead to complications, but treatment is effective.