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Autoimmune pancreatitis in children: Clinical and imaging characteristics in a large patient sample
Maria S Lopez Gonzalez1, Pradipta Debnath2, Nadeen K Abu Ata3
1Division of Pediatric Gastroenterology, Hepatology and Nutrition, Nicklaus Children's Hospital, Miami, Florida, USA.
Insights
Autoimmune pancreatitis (AIP) in children is often diagnosed using imaging, with magnetic resonance imaging (MRI) being key. Modest elevations in gamma-glutamyl transferase and direct bilirubin may indicate AIP, prompting MRI for diagnosis and steroid treatment.
Area of Science:
- Pediatric Gastroenterology
- Autoimmune Diseases
- Diagnostic Imaging
Background:
- Autoimmune pancreatitis (AIP) is a rare chronic inflammatory condition affecting the pancreas.
- Accurate diagnosis and understanding of clinical and imaging features in children are crucial for effective management.
- Previous studies have focused less on pediatric AIP, necessitating further research into its specific characteristics.
Purpose of the Study:
- To delineate the clinical presentation and characteristic imaging findings in a cohort of pediatric patients diagnosed with autoimmune pancreatitis.
- To evaluate the diagnostic utility of various methods, including imaging and serological markers, in identifying AIP in children.
- To assess the treatment response and outcomes in children with AIP.
Main Methods:
- Retrospective review of medical records for 40 pediatric patients with suspected AIP between January 2009 and August 2023.
- Analysis of demographic, clinical, histological, and laboratory data at initial presentation.
- Detailed review of imaging studies (primarily MRI) performed within 90 days of diagnosis by a pediatric radiologist.
Main Results:
- The majority of pediatric AIP cases (87.5%) were diagnosed based on imaging findings.
- Magnetic resonance imaging (MRI) revealed pancreas T1-weighted hypointensity in 90% of cases.
- Elevated gamma-glutamyl transferase (62.5%) and direct bilirubin (44%) were common, often without gallstones.
- Comorbidities included inflammatory bowel disease or other autoimmune conditions in 42.5% of patients.
- Steroid treatment led to symptom resolution in 87% of patients.
Conclusions:
- Imaging, particularly MRI, plays a pivotal role in diagnosing autoimmune pancreatitis in children.
- Slight elevations in GGT and direct bilirubin, in the absence of gallstones, should raise suspicion for AIP.
- Prompt diagnosis via MRI followed by appropriate steroid therapy is effective in managing pediatric AIP.
Objective:
To describe clinical and imaging findings of a large sample of children with autoimmune pancreatitis (AIP).
Methods:
We retrospectively reviewed medical records for patients with clinical features consistent with AIP from January 2009 to August 2023. Demographic, clinical, and histological findings on initial presentation were recorded. Imaging within 90 days of diagnosis was reviewed by a board-certified pediatric radiologist.
Results:
Forty patients were included. Mean age was 12.9 years and 23 (57.5%) were female. AIP diagnosis was based on imaging in 87.5% (35/40), only one patient (2.5%) had all three diagnostic features (imaging, biopsy, immunoglobulin G4 [IgG4]). Two diagnostic features were present in 27.5% (11/40) of patients (15% imaging & IgG4, 12.5% imaging and biopsy). Gamma-glutamyl transferase (GGT) elevation was present in 62.5% (20/32) of patients and elevated direct bilirubin was present in 44% (11/25). Magnetic resonance imaging (MRI) was the most common imaging modality (75% of patients, 30/40) and the most common finding was pancreas T1-weighted hypointensity (90%, 26/29). Inflammatory bowel disease or another autoimmune condition was ultimately diagnosed in 42.5% of patients (17/40). Most patients (79%, 31/39) were treated with steroids over a 2-3-month period, resulting in symptom resolution in 87% (26/30).
Conclusion:
In 40 patients with features of AIP, most were diagnosed by imaging. A modest elevation of GGT and direct bilirubin in the absence of cholelithiasis might suggest AIP in the appropriate clinical context and should prompt consideration of MRI for diagnosis, followed by proper treatment.
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