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Pediatric Autoimmune Pancreatitis: A Single-Center Retrospective Case Series
Erica C Becker1, Rakesh Kumar2, Adnan Altaf2
1From the Department of Internal Medicine, University of Connecticut Health Center, Farmington, CT.
Insights
Autoimmune pancreatitis (AIP) is rare in children, presenting with pain and jaundice. Steroid treatment showed prompt symptom resolution, indicating a good prognosis for this pediatric condition.
Area of Science:
- Pediatric Gastroenterology
- Immunology
- Hepatology
Background:
- Autoimmune pancreatitis (AIP) is a rare immune-mediated pancreatic condition.
- Limited data exists on pediatric AIP presentation and outcomes.
Purpose of the Study:
- To describe the presentation, symptoms, and outcomes of pediatric AIP.
- To enhance understanding of this rare condition in children.
Main Methods:
- Single-center case series of 4 pediatric patients.
- Diagnosis based on serology, imaging, histology, and steroid response.
- Data included demographics, symptoms, imaging, organ involvement, histology, treatment, and outcomes.
Main Results:
- All patients had type 2 AIP with abdominal pain, emesis, and jaundice.
- Imaging revealed pancreatic enlargement and bile duct strictures.
- Biopsies showed lymphoplasmacytic infiltration and fibrosis.
- Prednisone treatment led to symptom and lab normalization.
Conclusions:
- Pediatric AIP is rare with distinct clinical, radiographic, and histological features.
- Type 2 AIP in children shows a good long-term prognosis.
- Prompt response to steroid therapy observed.
Objectives:
Autoimmune pancreatitis (AIP) is a rare form of a chronic, immune-mediated, inflammatory pancreatic condition. There is limited data regarding presentation and outcome in the pediatric population. We described a single-center case series of 4 pediatric patients with AIP to better understand the presentation, symptoms, and outcomes of this rare condition.
Methods:
Data collected included demographics, serology markers, symptoms at presentation, imaging, additional organ involvement, histology, treatment methods, and outcomes. The diagnosis of AIP was made by a combination of serology, pancreatic imaging, histology, extrapancreatic manifestations, and steroid response.
Results:
All patients were diagnosed with type 2 AIP. Abdominal pain, emesis, and obstructive jaundice were the most common symptoms at presentation. Autoimmune markers were negative. Cross-sectional abdominal imaging showed diffuse/focal pancreatic gland enlargement and common bile duct stricture universally and main pancreatic duct irregularity in half of the patients. Biopsies showed lymphoplasmacytic infiltration with associated pancreatic fibrosis and atrophy. Treatment with an 8-week tapering course of prednisone showed prompt response with resolution of symptoms and normalization of laboratory test results.
Conclusions:
Our case series shows that AIP in children is a rare entity with a distinct clinical presentation, classical radiographic and histological features with good long-term prognosis.
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