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Published on: December 14, 2019
Autoimmune pancreatitis in children: A single centre experience in diagnosis, management and long term follow up
Huey Miin Lee1, Maesha Deheragoda2, Phil Harrison2
1Paediatric Liver, GI & Nutrition Centre and MowatLabs, King's College Hospital NHS Foundation Trust, London, UK.
Insights
This study of pediatric autoimmune pancreatitis (AIP) found a good long-term prognosis in a small cohort. Further research is needed to fully understand this rare condition in children.
Area of Science:
- Gastroenterology
- Pediatric Medicine
- Immunology
Background:
- Autoimmune pancreatitis (AIP) is a rare chronic inflammatory condition affecting the pancreas.
- Limited data exists regarding pediatric AIP, necessitating further characterization.
Purpose of the Study:
- To detail the presentation, diagnosis, management, and long-term outcomes of pediatric patients with AIP.
- To contribute to a better understanding of AIP in the pediatric population.
Main Methods:
- Retrospective review of data from six pediatric patients diagnosed with AIP over a 10-year period.
- Collected data included demographics, clinical information, laboratory and serological markers, radiological and histological findings, and follow-up data.
Main Results:
- One patient had Type 1 AIP, while five had Type 2 AIP (definitive, probable, or suspected).
- Median follow-up was 3.9 years. Four patients underwent pancreatic biopsies, with two showing granulocytic epithelial lesions (GELs).
- Four patients received steroid treatment; two developed ulcerative colitis. Two patients experienced exocrine insufficiency.
Conclusions:
- The long-term prognosis for pediatric AIP in this cohort appears favorable.
- Additional follow-up data is crucial for further defining and characterizing pediatric AIP.
Objectives:
Autoimmune pancreatitis (AIP) 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 AIP 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 AIP 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 AIP, two with definitive Type 2 AIP, two with probable Type 2 AIP and one with suspected Type 2 AIP. 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 AIP 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 AIP shows good prognosis. More follow up data on patients with AIP is needed to help further characterize and define the disease.
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