Autoimmune pancreatitis in children: A single centre experience in diagnosis, management and long term follow up

Objectives: Autoimmune pancreatitis (ALP) is a rare form of chronic pancreatitis and data is limited in the paediatric population. We aim to describe in detail a cohort of paediatric patients with ALP including their presentation, investigations that led to their diagnosis, management and long-term follow up. Methods: We retrospectively reviewed the data of 6 patients diagnosed with ALP over an 10-year period. Data including demographics, clinical information, laboratory parameters, serological markers, radiological and histological findings as well as longitudinal follow up were collected. Results: Out of the six patients, one was diagnosed with definitive Type 1 ALP, two with definitive Type 2 ALP, two with probable Type 2 ALP and one with suspected Type 2 ALP. Median time of follow up was 3.9 years (range 2.6-10.1). 4 patients had pancreatic biopsies with 2 of these patients showing granulocytic epithelial lesions (GELs). 4 patients received steroids and two of them developed ulcerative colitis. Azathioprine was commenced on the patient with Type 1 ALP to help her wean off steroids that caused significant side effects on her. Only two patients developed exocrine insufficiency. Conclusions: The long term follow up of our cohort of paediatric ALP shows good prognosis. More follow up data on patients with ALP is needed to help further characterize and define the disease. Crown Copyright (C) 2018 Published by Elsevier B.V. on behalf of IAP and EPC. All rights reserved.